RESUMEN
Hypertension does not recognize obvious pathogenic causes in the majority of patients (essential hypertension). However, a secondary underlying cause of hypertension can be recognized in 5-10% of unselected hypertensive patients, and this prevalence may increase to more than 20% in patients with hypertension that is difficult to control or frankly resistant to treatment. In children, secondary hypertension is most often due to aortic coarctation, distal thoracic or abdominal aortic stenosis, or specific gene mutations. In adults or elderly individuals, secondary hypertension is most often due to atherosclerotic renal artery stenosis, primary hyperaldosteronism, and Cushing's disease or syndrome. Parenchymal nephropathy and hyperparathyroidism can cause hypertension at all ages, while pheochromocytoma and paraganglioma tend to occur more often in adolescents or young adults. In general, secondary hypertension should be suspected in subjects with: (a) onset of hypertension under 30 years of age especially if in the absence of hypertensive family history or other risk factors for hypertension; (b) treatment-resistant hypertension; c) severe hypertension (>180/110 mmHg), malignancy, or hypertensive emergencies; d) rapid rise in blood pressure values in previously well controlled patients. Any clinical signs suspicious or suggestive of hypertension from endocrine causes, a "reverse dipping" or "non-dipping'" profile at 24 h ambulatory blood pressure monitoring not justified by other factors, signs of obvious organ damage may be helpful clues for diagnosis. Finally, patients snoring or with clear sleep apnea should also be considered for possible secondary hypertension.
Asunto(s)
Hipertensión , Humanos , Hipertensión/etiología , Hipertensión/diagnóstico , Adulto , Hiperaldosteronismo/diagnóstico , Hiperaldosteronismo/complicaciones , Hiperaldosteronismo/terapia , Niño , Antihipertensivos/uso terapéutico , Coartación Aórtica/diagnóstico , Coartación Aórtica/complicaciones , Coartación Aórtica/terapiaRESUMEN
BACKGROUND: Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart defect during prenatal screening. High false positive and false negative rates seriously affect prenatal consultation and postnatal management. The objective of the study was to assess the utility of various measurements to predict prenatal CoA and to derive a diagnostic algorithm. METHODS: One hundred and fifty-four fetuses with suspected CoA who presented at Fuwai Hospital between December 2017 and August 2021 were enrolled and divided into confirmed CoA cases (n = 47) and false positive cases (n = 107), according to their postnatal outcomes. The transverse aortic arch, isthmus, and descending aorta were measured in the long-axis view of the aortic arch. The angle between the transverse aortic arch (TAO) and the descending aortic arch (DAO) was defined as the TAO-DAO angle and measured in the long axis or sagittal view. Based on the database in GE Voluson E10 and the formula (Z = [Formula: see text]), the standard score (Z-score) of the dimensions of the aorta were calculated in relation to the gestational age. The main echocardiographic indices were combined to design a 3-step diagnostic protocol. The TAO-DAO angle was used as the first step in the diagnostic model. The diameter of the transverse arch and the Z-score of the isthmus were the second step. The third-step indices included a Z-score of the transverse arch, diameter of the isthmus, distance from the left subclavian artery (LSA) to left common carotid artery (LCCA), the ratio of isthmus diameter and LSA diameter and ratio of the distances (the distance between the LSA and LCCA to the distance between the right innominate artery and LCCA). The receiver operating characteristic (ROC) curve determined the predictive capability of each diagnostic parameter, and the kappa test determined the diagnostic accuracy of the proposed model. RESULTS: The cases with confirmed CoA had thinner transverse arches (1.92 ± 0.32 mm vs. 3.06 ± 0.67 mm, P = 0.0001), lower Z-scores of the isthmus (-8.97 ± 1.45 vs. -5.65 ± 1.60, P = 0.0001), smaller TAO-DAO angles (105.54 ± 11.51° vs. 125.29 ± 8.97°, P = 0.0001) and larger distance between the LSA and LCCA (4.45 ± 1.75 mm vs. 2.74 ± 1.07 mm, P = 0.0001) than the false positive cases. The area under the curve (AUC) was 0.947 (95% CI 0.91-0.98) for the TAO-DAO angle ≤ 115.75°, 0.942 (95% CI 0.91-0.98) for the transverse arch diameter ≤ 2.31 mm, 0.937 (95% CI 0.90-0.98) for the Z-score of the isthmus ≤ -7.5, and 0.975 (95% CI 0.95-1.00) for the 3-step diagnostic protocol with 97.8% sensitivity and 97.2% specificity. The kappa test showed that the model's diagnostic accuracy was consistent with postnatal outcomes (kappa value 0.936, P = 0.0001). CONCLUSIONS: The 3-step diagnostic protocol included the three most useful measurements and the additional indices with appropriate cut-off values. The algorithm is useful for the detection of aortic coarctation in fetuses with a high degree of accuracy. TRIAL REGISTRATION: Retrospectively registered.
Asunto(s)
Aorta Torácica , Coartación Aórtica , Ecocardiografía , Ultrasonografía Prenatal , Humanos , Coartación Aórtica/diagnóstico por imagen , Femenino , Embarazo , Aorta Torácica/diagnóstico por imagen , Estudios Retrospectivos , Algoritmos , Edad GestacionalRESUMEN
OBJECTIVE: Shone's complex comprises of a combination of congenital cardiac anomalies causing obstructions in the left ventricle's inflow and outflow tracts. This systematic review aims to evaluate the clinical features and surgical outcomes of Shone's complex. METHODS: An electronic literature search of PubMed and Scopus was performed to identify relevant studies related to the presentation, management, and outcomes of Shone's complex. Two reviewers independently performed selection. Data on study characteristics, participant demographics, interventions, outcomes, and follow-up durations were extracted and analyzed. RESULTS: A total of 691 papers were identified, with 18 studies included in the final analysis. The majority of the studies (n = 12) focused on the pediatric age group. The most common clinical presentations were coarctation of the aorta (n = 17) and mitral stenosis (n = 12). Surgical interventions often involved staged approaches, prioritizing outflow before inflow obstructions. Mitral valve repair was preferred over replacement due to better long-term outcomes (n = 8). Biventricular repair was recommended due to improved postoperative outcomes, but often needed reoperations. Reoperations were common, primarily due to recurrent coarctation (n = 10), subaortic stenosis (n = 8), and mitral valve dysfunction (n = 7). Pulmonary hypertension (n = 10) and arrhythmias (n = 11) were significant complications. Most patients were in modified Ross/NYHA functional class 1 on follow-up. Mortality rates ranged from 4 to 28%, with better outcomes associated with early and strategic surgical interventions. CONCLUSION: Early diagnosis and biventricular repair were associated with better outcomes while transplantation was often an eventuality. Standardized diagnostic criteria, long-term follow-up, and consensus guidelines are needed to improve the management of this congenital heart disease.
Asunto(s)
Procedimientos Quirúrgicos Cardíacos , Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/complicaciones , Resultado del Tratamiento , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Lactante , Preescolar , Adolescente , Masculino , Femenino , Coartación Aórtica/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/mortalidad , Recién Nacido , Obstrucción del Flujo Ventricular Externo/cirugía , Obstrucción del Flujo Ventricular Externo/etiología , Reoperación , Factores de RiesgoRESUMEN
A girl in middle childhood presented with glaucoma in her right eye along with segmental haemangiomas on the right side of the face and neck. Magnetic resonance angiography of the brain showed hypoplasia of the right internal carotid artery, leading to the diagnosis of posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye abnormalities (PHACE) syndrome. High-definition anterior segment ocular coherence tomography (AS-OCT) of the right eye showed an absence of Schlemm's canal and a hyperreflective membrane over the trabecular meshwork. The presence of this angle dysgenesis on AS-OCT, a novel finding in this disease, explained the elevated intraocular pressure in the right eye. The embryological basis for the development of angle dysgenesis might help better understand the pathogenesis of PHACE syndrome.
Asunto(s)
Coartación Aórtica , Anomalías del Ojo , Glaucoma , Síndromes Neurocutáneos , Humanos , Femenino , Síndromes Neurocutáneos/diagnóstico por imagen , Síndromes Neurocutáneos/complicaciones , Síndromes Neurocutáneos/diagnóstico , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico , Anomalías del Ojo/diagnóstico por imagen , Anomalías del Ojo/complicaciones , Anomalías del Ojo/diagnóstico , Glaucoma/diagnóstico , Tomografía de Coherencia Óptica/métodos , Hemangioma/complicaciones , Hemangioma/diagnóstico por imagen , Arteria Carótida Interna/anomalías , Arteria Carótida Interna/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/complicaciones , Angiografía por Resonancia MagnéticaRESUMEN
Shone's syndrome (SS) is a rare congenital cardiac anomaly characterized by a spectrum of developmental abnormalities. It predominantly presents as consisting of a variety of left ventricular inflow and outflow tract lesions, with inflow tract lesions typically including parachute mitral valve and supravalvular mitral ring. However, reports of SS involving double-orifice mitral valve are scarce.
Asunto(s)
Coartación Aórtica , Estenosis de la Válvula Mitral , Válvula Mitral , Humanos , Estenosis de la Válvula Mitral/complicaciones , Estenosis de la Válvula Mitral/diagnóstico por imagen , Estenosis de la Válvula Mitral/cirugía , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/anomalías , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Masculino , Ecocardiografía/métodos , Anomalías Múltiples , Síndrome , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico por imagen , Diagnóstico DiferencialRESUMEN
BACKGROUND: A peculiar subgroup of patients with partial or complete atrioventricular canal defect exhibits a spectrum of left-sided obstructions including right ventricular dominance and aortic coarctation. The association of atrioventricular canal defect with left-sided obstructions is found in several genetic syndromes; however, the molecular basis of nonsyndromic atrioventricular canal defect with aortic coarctation is still poorly understood. Although some candidate genes for nonsyndromic atrioventricular canal defect are known, a complex oligogenic inheritance determined in some cases by the co-occurrence of multiple variants has also been hypothesized. CASE REPORT: We describe a nonsyndromic infant with mesocardia with viscero-atrial situs solitus, partial atrioventricular canal defect, mild right ventricular dominance, and coarctation of the aorta. Next generation sequencing genetic testing revealed variants in two genes, GDF1 and NOTCH1, previously reported in association with atrioventricular canal defect and left-sided obstructive lesions, respectively. CONCLUSION: The present report could support the hypothesis that the co-occurrence of cumulative variants may be considered as genetic predisposing risk factor for specific congenital heart defects.
Asunto(s)
Coartación Aórtica , Factor 1 de Diferenciación de Crecimiento , Receptor Notch1 , Humanos , Coartación Aórtica/genética , Receptor Notch1/genética , Factor 1 de Diferenciación de Crecimiento/genética , Defectos de los Tabiques Cardíacos/genética , Masculino , Femenino , Predisposición Genética a la Enfermedad , Recién Nacido , Mutación/genética , LactanteRESUMEN
The coarctation of the aorta (CoA) combined with heart defects or cerebral artery aneurysms is more prevalent in clinical practice. However, cases of concurrent bilateral iliac artery dissection (IAD) are extremely rare and have not been reported. Here, we described a case with CoA combined with bilateral IAD. The patient, a 62-year-old male, presented with acute intermittent claudication accompanied by pain and aching in both lower limbs after walking. Following a thorough medical history inquiry and examination, the patient was diagnosed with acute bilateral IAD combined with CoA. The patient underwent endovascular treatment. Postoperatively, the aortic diameter recovered, and the bilateral IAD disappeared, yielding satisfactory therapeutic results. Conclusively, endovascular treatment of aortic coarctation combined with IAD is an effective therapeutic approach, enhancing patient survival and improving their quality of life.
Asunto(s)
Coartación Aórtica , Disección Aórtica , Procedimientos Endovasculares , Humanos , Masculino , Persona de Mediana Edad , Disección Aórtica/complicaciones , Disección Aórtica/diagnóstico , Disección Aórtica/cirugía , Procedimientos Endovasculares/métodos , Coartación Aórtica/complicaciones , Coartación Aórtica/cirugía , Coartación Aórtica/diagnóstico , Arteria Ilíaca , Aneurisma Ilíaco/complicaciones , Aneurisma Ilíaco/cirugía , Aneurisma Ilíaco/diagnóstico , Claudicación Intermitente/etiología , Claudicación Intermitente/diagnóstico , Resultado del Tratamiento , Calidad de VidaRESUMEN
OBJECTIVES: Interruption of aortic arch (IAA) is a rare congenital heart disease. This study aims to investigate echocardiographic features and pathological ultrastructural characteristics of fetal IAA and to further analyze its pathological evolution. METHODS: A retrospective analysis was conducted on prenatal echocardiographic, post-surgical, or autopsy findings of fetuses prenatally diagnosed with IAA. Prenatal echocardiographic tracking was used to observe the internal diameters and Z-scores of different segments of the aortic arch and the changes in the narrowed section. These observations were combined with autopsy and pathological findings to explore the potential intrauterine evolution of IAA and its cytological basis. RESULTS: The study included 34 fetuses with IAA, with 3, 3, and 28 fetuses prenatally diagnosed with aortic arch dysplasia (AAD), coarctation of aorta (CoA), and IAA, respectively. The 3 AAD and 3 CoA fetuses chose termination of pregnancy 1 to 2 weeks after prenatal ultrasound diagnosis, and autopsy confirmed IAA. Among the 28 fetuses prenatally diagnosed with IAA, 6 cases of CoA progressively worsened, eventually evolving into type A IAA as observed through echocardiographic follow-up. The remaining 22 cases were diagnosed as IAA on the first prenatal ultrasound. Postnatal surgery corrected 3 cases, while 27 cases opted for pregnancy termination, and 4 cases resulted in intrauterine death. Echocardiographic features of the fetal IAA included a significantly smaller left ventricle compared with the right or negligible difference on the four-chamber view, a significantly smaller aorta than the pulmonary artery on the three-vessel view, and a lack of connection between the aorta and the descending aorta on the three-vessel-trachea and aortic arch views. The aortic arch appears less curved and more rigid, losing the normal "V" shape between the aorta, ductus arteriosus, and descending aorta. Color Doppler ultrasound showed no continuous blood flow signal at the interruption site, with reversed blood flow visible in the ductus arteriosus. Transmission electron microscopy of 7 IAA fetuses revealed numerous disorganized smooth muscle cells between the elastic membranes near the aortic arch interruption site, significantly increased in number compared with the proximal ascending aorta. The elastic membranes were thicker and more twisted near the interruption site. The interruption area lacked normal endothelial cells and lumen, with only remnants of necrotic endothelial cells, disorganized short and thick elastic membranes, and randomly arranged smooth muscle cells. CONCLUSIONS: Prenatal echocardiography is the primary diagnostic tool for fetal IAA. Post-surgical follow-up and autopsy help identify complications and disease characteristics, enhancing diagnostic accuracy. Some fetal IAA may evolve from AAD or CoA, with potential pathogenesis related to ischemia, hypoxia, and migration of ductal constrictive components.
Asunto(s)
Aorta Torácica , Ultrasonografía Prenatal , Humanos , Femenino , Aorta Torácica/embriología , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/patología , Embarazo , Estudios Retrospectivos , Ecocardiografía , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/patología , Cardiopatías Congénitas/embriología , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/patología , Coartación Aórtica/embriología , AdultoRESUMEN
BACKGROUND: Extra-anatomic ascending-to-descending aortic bypass grafts have historically been utilized as a safe and effective solution for repairs of complex coarctation of the aorta. However, reports on reoperation in these patients remain rare. We present a case of an aortic valve replacement and coronary artery bypass grafting in a patient with an extra-anatomic ascending-to-descending aortic bypass graft. CASE PRESENTATION: The patient is a 59-year-old male with a complex aortic history, including repair of aortic coarctation with an ascending-to-descending aortic bypass graft 13 years prior, was admitted to the hospital for shortness of breath and chest pain that had developed over the past year. On further workup, he was found to have severe bileaflet aortic valve stenosis, non-ST elevation myocardial infarction, and moderate coronary artery disease. He underwent surgical aortic valve replacement and coronary artery bypass grafting. Given his unique anatomy, cardiopulmonary bypass approach involved separate cannulation of the right axillary and left common femoral arteries with cross-clamp of both the aorta and the extra-anatomic graft. Using this approach, the redo operation was successfully performed. CONCLUSIONS: Reports on reoperation after ascending-to-descending aortic bypass grafting are rare. We describe our approach to cardiopulmonary bypass and reoperation in a patient with an extra-anatomic ascending-to-descending aortic bypass graft.
Asunto(s)
Puente de Arteria Coronaria , Reoperación , Humanos , Masculino , Persona de Mediana Edad , Puente de Arteria Coronaria/métodos , Implantación de Prótesis de Válvulas Cardíacas/métodos , Coartación Aórtica/cirugía , Aorta/cirugía , Estenosis de la Válvula Aórtica/cirugía , Válvula Aórtica/cirugíaRESUMEN
Mid-aortic syndrome (MAS) is a rare vascular disease that usually leads to renovascular hypertension. With the predominant manifestations being intractable arterial hypertension and lower extremity arterial insufficiency, it has rarely been associated with dilated cardiomyopathy. We report a young girl with congestive heart failure, where the cause was initially attributed to dilated cardiomyopathy. A repeated echocardiogram 6 months later brought the physician's suspicion of MAS because of the abnormal colour of Doppler from the subcostal view. Further assessment using CT angiography revealed discrete thoracic coarctation at the level of T10, with the narrowest diameter of 2.1 mm, thus confirming the diagnosis. Her inflammatory markers and connective tissue screening were negative. She underwent successful stenting of coarctation of the aorta, which later caused improvement in her cardiac function. We highlighted the importance of looking for treatable causes of dilated cardiomyopathy and vigilant clinical and echocardiogram assessment with high suspicion to diagnose MAS.
Asunto(s)
Coartación Aórtica , Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/complicaciones , Femenino , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico , Coartación Aórtica/diagnóstico por imagen , Angiografía por Tomografía Computarizada , Ecocardiografía , Stents , Diagnóstico Diferencial , Síndrome , Insuficiencia Cardíaca/etiologíaRESUMEN
BACKGROUND: Although coarctation of the aorta without concomitant intracardiac pathology is relatively common, there is lack of guidance regarding aspects of its management in neonates and infants. METHODS: A panel of experienced congenital cardiac surgeons, cardiologists, and intensivists was created, and key questions related to the management of isolated coarctation in neonates and infants were formed using the PICO (Patients/Population, Intervention, Comparison/Control, Outcome) Framework. A literature search was then performed for each question. Practice guidelines were developed with classification of recommendation and level of evidence using a modified Delphi method. RESULTS: For neonates and infants with isolated coarctation, surgery is indicated in the absence of obvious surgical contraindications. For patients with risk factors for surgery, medical management before intervention is reasonable. For those stable off prostaglandin E1, the threshold for intervention remains unclear. Thoracotomy is indicated when arch hypoplasia is not present. Sternotomy is preferable when arch hypoplasia is present that cannot be adequately addressed through a thoracotomy. Sternotomy may also be considered in the presence of a bovine aortic arch. Antegrade cerebral perfusion may be reasonable when the repair is performed through a sternotomy. Extended end-to-end, arch advancement, and patch augmentation are all reasonable techniques. CONCLUSIONS: Surgery remains the standard of care for the management of isolated coarctation in neonates and infants. Depending on degree and location, arch hypoplasia may require a sternotomy approach as opposed to a thoracotomy approach. Significant opportunities remain to better delineate management in these patients.
Asunto(s)
Coartación Aórtica , Humanos , Coartación Aórtica/cirugía , Recién Nacido , Lactante , Sociedades Médicas , Cirugía TorácicaRESUMEN
BACKGROUND: This study's objective is to describe outcomes of adult patients who underwent thoracic stent graft placement treatment for primary or recurrent aortic coarctation. METHODS: This is a retrospective study of 30 adult patients who underwent thoracic stent graft placement for aortic coarctation at our institution. Average age was 46.5 years, with 53.3% of patients presented with no prior treatment or repair for coarctation. Indications for repair included gradient ≥20 mm Hg with anatomic evidence of coarctation on imaging with left ventricular hypertrophy, pseudoaneurysm, aneurysm, refractory hypertension, or claudication. Stent grafts used for repair included MDT (Medtronic, Santa Rosa, CA) and GORE TAG (W. L. Gore & Associates, Flagstaff, AZ). RESULTS: Patients were observed for a median of 979 days, with one death during the study. All patients had complete resolution of symptoms with no recurrences. Thoracic endovascular aortic repair significantly reduced the gradient across the coarctation (P < 0.0001). Aortic coarctation diameter significantly increased at 30 days postoperatively and continued to increase up to 5 years posttreatment. At 3+ years, aortic remodeling was observed at the coarctation site and surrounding regions. At 30 days, systolic, diastolic, and mean arterial pressure were all reduced. Systolic and diastolic blood pressure and mean arterial pressure continued to significantly improve 1-year posttreatment. CONCLUSIONS: Stent grafts are a safe and effective treatment for aortic coarctation. We observed a clinically significant improvement in blood pressure and longitudinal aortic remodeling of the coarctation segment and the entire aorta that persisted more than more than 3 years.
Asunto(s)
Aorta Torácica , Coartación Aórtica , Implantación de Prótesis Vascular , Prótesis Vascular , Procedimientos Endovasculares , Diseño de Prótesis , Stents , Remodelación Vascular , Humanos , Coartación Aórtica/cirugía , Coartación Aórtica/fisiopatología , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/complicaciones , Estudios Retrospectivos , Masculino , Femenino , Persona de Mediana Edad , Resultado del Tratamiento , Adulto , Procedimientos Endovasculares/instrumentación , Procedimientos Endovasculares/efectos adversos , Implantación de Prótesis Vascular/instrumentación , Implantación de Prótesis Vascular/efectos adversos , Factores de Tiempo , Aorta Torácica/cirugía , Aorta Torácica/fisiopatología , Aorta Torácica/diagnóstico por imagen , Recurrencia , Anciano , Adulto JovenRESUMEN
OBJECTIVE: This study sought to elucidate clinical and imaging findings predictive for malperfusion syndrome after blunt thoracic aortic injury (BTAI). BACKGROUND: There is limited literature on malperfusion syndrome after BTAI, and the timing of thoracic endovascular aortic repair (TEVAR) in patients with this condition has not been defined. METHODS: A retrospective analysis of prospectively collected data of patients with BTAI treated between January 2021 and October 2023. Clinical and thoracic aortic (TA) imaging data, time to TEVAR, in-hospital death, and malperfusion/reperfusion sequelae (paraplegia, renal/visceral/limb ischemia, and compartment syndromes) were assessed. Correlations between clinical and imaging findings, time to TEVAR, and outcomes were evaluated. RESULTS: Of the 19,203 trauma patients evaluated, 13,717 (71%) had blunt injuries and 77 (0.6%) had BTAI. The majority (67.5%) were male, with a median age of 40 years (IQR: 33-55). TEVAR was performed in 42 (54.5%) patients. Seven (9.1%) patients presented with clinical and TA imaging criteria for traumatic thoracic aortic coarctation (TTAC), including diminished/absent femoral pulses and TA luminal narrowing of 50% to 99%. The median time to TEVAR was 9 (IQR: 5-32), 11, and 4 hours for all non-TTAC and TTAC BTAI patients, respectively ( P =0.037). Only TTAC patients presented/developed malperfusion/reperfusion sequelae. In-hospital mortality rates were 7.8%, 5.8%, and 29% for all non-TTAC and TTAC BTAI patients, respectively ( P =0.09). Aortic-related mortality occurred in only 2 (2.6%) TTAC patients. CONCLUSIONS: Patients with clinical and TA imaging manifestations of TTAC are predisposed to malperfusion/reperfusion sequelae if TEVAR is delayed. We recommend the emergent repair of all BTAIs with TTAC.
Asunto(s)
Aorta Torácica , Coartación Aórtica , Procedimientos Endovasculares , Traumatismos Torácicos , Heridas no Penetrantes , Humanos , Masculino , Heridas no Penetrantes/complicaciones , Heridas no Penetrantes/cirugía , Femenino , Procedimientos Endovasculares/métodos , Aorta Torácica/lesiones , Aorta Torácica/cirugía , Aorta Torácica/diagnóstico por imagen , Estudios Retrospectivos , Adulto , Persona de Mediana Edad , Coartación Aórtica/cirugía , Traumatismos Torácicos/complicaciones , Traumatismos Torácicos/cirugía , Mortalidad Hospitalaria , Reparación Endovascular de AneurismasRESUMEN
BACKGROUND: The purpose of this study was to review echocardiography-based diagnosis of persistent fifth aortic arch (PFAA) in children. METHODS: From January 2015 to December 2022, we retrospectively analyzed the echocardiographic findings and the relevant clinical data during follow-up of patients with PFAA who were treated in the Third Affiliated Hospital of Zhengzhou University. The diagnosis was confirmed by computed tomography angiography or surgery. RESULTS: Seven PFAA cases included two Weinberg type A and five Weinberg type B. The anatomical details of PFAA were assessed using a combination of the long-axis view of the left ventricular outflow tract (from the left high parasternal window) and the long-axis view of the aortic arch (from the suprasternal window). In Weinberg type A, the distal fifth and fourth aortic arches were connected to the descending aorta, which was associated with aortic coarctation. In Weinberg type B, the upper arch of the fourth aorta was interrupted, and only the lower arch of the fifth aorta was connected to the descending aorta. Surgical repair of PFAA was indicated in five patients with blood flow disruption, among which four had good postoperative results and one refused surgery. Two patients with unobstructed PFAA blood flow required follow-up rather than surgery. CONCLUSIONS: It is feasible to diagnose PFAA by echocardiography. Combined application of the high parasternal left ventricular outflow tract view and the suprasternal aortic arch view can improve timely detection of different types of PFAA in children.
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Aorta Torácica , Angiografía por Tomografía Computarizada , Valor Predictivo de las Pruebas , Humanos , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/anomalías , Aorta Torácica/cirugía , Estudios Retrospectivos , Masculino , Femenino , Lactante , Preescolar , Aortografía , Niño , China , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/cirugía , Resultado del Tratamiento , Factores de Edad , Reproducibilidad de los Resultados , EcocardiografíaRESUMEN
BACKGROUND: A left thoracotomy approach is anatomically appropriate for childhood aortic coarctation; however, the pediatric femoral arteriovenous diameters are too small for cardiopulmonary bypass cannulation. We aimed to determine the safety of a partial cardiopulmonary bypass through the main pulmonary artery and the descending aorta in pediatric aortic coarctation repair. METHODS: We retrospectively reviewed 10 patients who underwent coarctation repair under partial main pulmonary artery-to-descending aorta cardiopulmonary bypass with a left thoracotomy as the CPB group. During the same period, 16 cases of simple coarctation of the aorta repair, with end-to-end anastomosis through a left thoracotomy without partial CPB assistance, were included as the non-CPB group to evaluate the impact of partial CPB. RESULTS: The median age and weight at surgery of the CPB group were 3.1 years (range, 9 days to 17.9 years) and 14.0 (range, 2.8-40.7) kg, respectively. Indications for the partial cardiopulmonary bypass with overlap were as follows: age > 1 year (n = 7), mild aortic coarctation (n = 3), and predicted ischemic time > 30 min (n = 5). Coarctation repair using autologous tissue was performed in seven cases and graft replacement in three. The mean partial cardiopulmonary bypass time, descending aortic clamp time, and cardiopulmonary bypass flow rate were 73 ± 37 min, 57 ± 27 min, and 1.6 ± 0.2 L/min/m2, respectively. Urine output during descending aortic clamping was observed in most cases in the CPB group (mean: 9.1 ± 7.9 mL/kg/h), and the total intraoperative urine output was 3.2 ± 2.7 mL/kg/h and 1.2 ± 1.5 mL/kg/h in the CPB and non-CPB group, respectively (p = 0.020). The median ventilation time was 1 day (range, 0-15), and the intensive care unit stay duration was 4 days (range, 1-16) with no surgical deaths. No major complications, including paraplegia or recurrent coarctation, occurred postoperatively during a median observation period of 8.1 (range, 3.4-17.5) years in the CPB group. In contrast, reoperation with recurrent coarctation was observed in 2 cases in the non-CPB group (p = 0.37). CONCLUSIONS: Partial cardiopulmonary bypass through the main pulmonary artery and descending aorta via a left thoracotomy is a safe and useful option for aortic coarctation repair in children.
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Coartación Aórtica , Puente Cardiopulmonar , Toracotomía , Humanos , Coartación Aórtica/cirugía , Estudios Retrospectivos , Puente Cardiopulmonar/métodos , Preescolar , Niño , Lactante , Toracotomía/métodos , Masculino , Femenino , Adolescente , Recién Nacido , Aorta Torácica/cirugía , Arteria Pulmonar/cirugía , Resultado del TratamientoRESUMEN
Generalized arterial calcification of infancy (GACI) is a rare genetic condition with varied clinical presentation. Consequently, diagnosis is frequently delayed or missed. GACI has a poor prognosis, with more than half of patients dying before the age of 6 months. Early diagnosis and treatment with bisphosphonates have been shown to improve survival in these patients. This is a case report of a newborn with respiratory distress who was initially diagnosed with coarctation of the aorta at echocardiography. Further imaging with CT revealed the aortic narrowing to be associated with GACI. Keywords: Genetic Defects, Congenital, Vascular, Calcification/Calculi, Aorta, Pulmonary Arteries, CT Angiography, Echocardiography, Pediatrics © RSNA, 2024.
Asunto(s)
Coartación Aórtica , Ecocardiografía , Calcificación Vascular , Humanos , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/diagnóstico , Recién Nacido , Calcificación Vascular/diagnóstico por imagen , Calcificación Vascular/diagnóstico , Diagnóstico Diferencial , Masculino , Angiografía por Tomografía Computarizada , FemeninoRESUMEN
Objectives: The configuration of the aortic arch, particularly a Gothic arch shape, in individuals with corrected coarctation of the aorta (CoA) has been associated with a decreased systolic wave amplitude across the arch, which could potentially impair renal perfusion and elevate the risk of arterial hypertension. This study aims to explore the relationship between the morphological characteristics of the aortic arch and their impact on renal perfusion in patients with CoA. Methods: Seventy-one subjects with corrected CoA underwent continuous 24 h ambulatory blood pressure monitoring, computed tomography to assess the aortic arch, and renal perfusion scanning. Subjects were stratified into three groups based on the height-to-width (H/W) ratio of their aortic arch: Group 1 with a H/W ratio of <0.65, Group 2 with a H/W ratio between 0.65 and 0.85, and Group 3 with a H/W ratio of >0.85. Results: Groups 1 and 2 (53,78% and 62.63%) presented with a higher hypertension prevalence of elevated blood pressure than Group 3 (38.89%). Notable variations were observed among the subjects in the time to peak perfusion (Tmax) in the left kidney across the groups. Group 1 showed a median Tmax at 0.27, Group 2 at 0.13, and Group 3 at -0.38 (p-value = 0.079). The differences in Tmax for the right kidney followed a similar trend but were not statistically significant (Group 1 at 0.61, Group 2 at 0.22, and Group 3 at 0.11; p-value = 0.229). Conclusions: This study suggests that variations in the aortic arch morphology might not significantly influence renal perfusion in CoA patients. This indicates the potential adaptability of the renal blood flow, which appears to compensate for reduced perfusion, thus minimizing adverse effects on the kidney function. This adaptability suggests an inherent physiological resilience, emphasizing the need for further targeted research to understand the specific interactions and impacts on treatment strategies for CoA.
Asunto(s)
Aorta Torácica , Coartación Aórtica , Humanos , Coartación Aórtica/fisiopatología , Coartación Aórtica/diagnóstico por imagen , Femenino , Masculino , Adulto , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/fisiopatología , Hipertensión/fisiopatología , Hipertensión/complicaciones , Riñón/fisiopatología , Riñón/irrigación sanguínea , Riñón/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Monitoreo Ambulatorio de la Presión Arterial/métodos , Persona de Mediana Edad , AdolescenteRESUMEN
Advancements in early diagnosis and paediatric cardiac surgery have improved the long-term survival of patients with congenital heart disease, necessitating a thorough assessment of their health-related quality of life (HRQoL). This study aimed to assess HRQoL in paediatric patients with coarctation of the aorta (CoA) (both as reported by patients and caregivers), and to evaluate associated factors. Patients aged 5-18 years diagnosed with CoA and their parents were enrolled at Bambino Gesù Children's Hospital between September 2016 and December 2017. Socio-demographic characteristics were recorded using a family form, and the Pediatric Quality of Life Inventory (PedsQL) 3.0 cardiac module was used to evaluate HRQoL. Clinical data were retrieved from medical chart reviews. In this observational study, sixty-five pediatric patients (39 males, median [IQR] age 12 [9-14]) with CoA and their parents (65 mothers and 65 fathers) were enrolled. These patients exhibited overall good HRQoL. Mothers reported significantly lower total HRQoL scores compared to patient self-reports (p = .037), as well as treatment anxiety (p = .033), and cognitive problems (p = .021). Pediatric patients with CoA perceived their HRQoL better than their mothers did. Female sex and older age were associated with lower HRQoL scores.
Asunto(s)
Coartación Aórtica , Calidad de Vida , Humanos , Masculino , Calidad de Vida/psicología , Femenino , Coartación Aórtica/cirugía , Coartación Aórtica/psicología , Niño , Adolescente , PreescolarRESUMEN
The present study aimed to evaluate the outcomes of percutaneous treatment of aortic coarctation using self-expandable uncovered Nitinol stents. We conducted a retrospective clinical data review of all patients with aortic coarctation and treated with self-expandable uncovered Nitinol stents at our institution between 2009 and 2019. The gradient pressure across the coarctation site was measured using aortography. Follow-up echocardiography and computed tomography angiography were performed to assess possible stent complications. A total of 127 stents were successfully implanted in 125 patients (64.8% males) with a mean age of 35.36 ± 11.9 years. The gradient across the coarctation site decreased significantly from 67.48 ± 14.79 to 5.04 ± 3.01 mmHg (P < 0.001) after self-expandable stent implantation. Systolic blood pressure (SBP) decreased significantly from 175.53 ± 15.99 to 147.22 ± 12.83 mmHg (P < 0.001) after self-expandable stenting. There were no major technical or clinical complications, including balloon rupture, aneurysmal formation, infection, secondary stent migration, thrombosis, death during the procedure, and in-hospital mortality. On a mean follow-up of 48 ± 23.6 months (12-120 months), the gradient [from 59.43 ± 15.42 to 3.72 ± 1.38 mmHg (P < 0.001)] and SBP [from 175.53 ± 15.99 to 127.99 ± 7.82 mmHg (P < 0.001)] decreased significantly. There was no mortality, aneurysmal formation in the stent site, dislocation, or aortic re-stenosis requiring intervention during mid-term follow-up. Treatment of aortic coarctation using a self-expandable uncovered nitinol stent is safe and effective with promising mid-term outcomes.