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
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
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
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: 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
PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as "ghost teeth", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child's dentition as it develops.
Asunto(s)
Coartación Aórtica , Anomalías del Ojo , Síndromes Neurocutáneos , Odontodisplasia , Humanos , Masculino , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Odontodisplasia/diagnóstico por imagen , Anomalías del Ojo/complicaciones , Preescolar , Síndromes Neurocutáneos/complicaciones , Fosa Craneal Posterior/anomalías , Fosa Craneal Posterior/diagnóstico por imagen , Extracción DentalRESUMEN
OBJECTIVE: This study aims to compare aortic morphology between repaired coarctation patients and controls, and to identify aortic morphological risk factors for hypertension and cardiovascular events (CVEs) in coarctation patients. METHODS: Repaired coarctation patients with computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were included, followed-up and compared with sex-matched and age-matched controls. Three-dimensional aortic shape was reconstructed using patients' CTA or MRA, or four-dimensional flow cardiovascular magnetic resonance in controls, and advanced geometrical characteristics were calculated and visualised using statistical shape modelling. In patients, we examined the association of geometrical characteristics with (1) baseline hypertension, using multivariable logistic regression; and (2) cardiovascular events (CVE, composite of aortic complications, coronary artery disease, ventricular arrhythmias, heart failure hospitalisation, stroke, transient ischaemic attacks and cardiovascular death), using multivariable Cox regression. The least absolute shrinkage and selection operator (LASSO) method selected the most informative multivariable model. RESULTS: Sixty-five repaired coarctation patients (23 years (IQR 19-38)) were included, of which 44 (68%) patients were hypertensive at baseline. After a median follow-up of 8.7 years (IQR 4.8-15.4), 27 CVEs occurred in 20 patients. Aortic arch dimensions were smaller in patients compared with controls (diameter p<0.001, wall surface area p=0.026, volume p=0.007). Patients had more aortic arch torsion (p<0.001) and a higher curvature (p<0.001). No geometrical characteristics were associated with hypertension. LASSO selected left ventricular mass, male sex, tortuosity and age for the multivariable model. Left ventricular mass (p=0.014) was independently associated with CVE, and aortic tortuosity showed a trend towards significance (p=0.070). CONCLUSION: Repaired coarctation patients have a smaller aortic arch and a more tortuous course of the aorta compared with controls. Besides left ventricular mass index, geometrical features might be of importance in long-term risk assessment in coarctation patients.
Asunto(s)
Coartación Aórtica , Angiografía por Tomografía Computarizada , Angiografía por Resonancia Magnética , Humanos , Coartación Aórtica/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Masculino , Femenino , Angiografía por Tomografía Computarizada/métodos , Adulto , Factores de Riesgo , Adulto Joven , Estudios de Seguimiento , Factores de Tiempo , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/cirugía , Estudios Retrospectivos , Imagen por Resonancia Cinemagnética/métodos , Medición de Riesgo/métodos , Resultado del Tratamiento , Hipertensión/complicaciones , Hipertensión/fisiopatología , AdolescenteRESUMEN
Various surgical techniques have been developed for coarctation of the aorta. However, coarctation repair in neonates with arch hypoplasia remains challenging. We herein report a case in which a premature neonate under 1500â g with coarctation of the aorta and arch hypoplasia underwent an extra-anatomical bypass at 18 days old. A second extra-anatomical bypass was performed at 3 years of age, and a third extra-anatomic bypass for recurrent coarctation was performed in adulthood. By increasing the size of the graft as the patient grows, extra-anatomic bypass can be a useful surgical option for premature neonates with coarctation and arch hypoplasia.
Asunto(s)
Aorta Torácica , Coartación Aórtica , Implantación de Prótesis Vascular , Humanos , Coartación Aórtica/cirugía , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/complicaciones , Recién Nacido , Aorta Torácica/cirugía , Aorta Torácica/anomalías , Aorta Torácica/diagnóstico por imagen , Resultado del Tratamiento , Implantación de Prótesis Vascular/instrumentación , Masculino , Recien Nacido Prematuro , Aortografía , Recurrencia , Prótesis Vascular , Angiografía por Tomografía Computarizada , Factores de Edad , Edad Gestacional , Preescolar , Adulto , FemeninoAsunto(s)
Coartación Aórtica , Síndrome de Turner , Humanos , Síndrome de Turner/complicaciones , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Femenino , Aneurisma de la Aorta/diagnóstico por imagen , Aneurisma de la Aorta/complicaciones , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/anomalías , Adulto , Aneurisma de la Raíz de la AortaAsunto(s)
Aorta Torácica , Coartación Aórtica , Imagen Multimodal , Humanos , Masculino , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/anomalías , Aorta Torácica/cirugía , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/diagnóstico , Coartación Aórtica/complicaciones , Coartación Aórtica/cirugía , Aortografía/métodos , Angiografía por Tomografía ComputarizadaRESUMEN
BACKGROUND: Aortic aneurysm is common in patients with coarctation of aorta (COA), but it is unclear whether the risk of aortic aneurysms is due to COA or related to the presence of other risk factors such as bicuspid aortic valve (BAV) and hypertension. OBJECTIVES: The purpose of this study was to assess the relationship among COA, BAV, and thoracic aortic aneurysms. METHODS: A total of 867 patients with COA (COA group) were matched 1:1:1 to 867 patients with isolated BAV (BAV group) and 867 patients without structural heart disease (SHD) (no-SHD group). The COA group was further subdivided into a COA+BAV subgroup (n = 304 [35%]), and COA with tricuspid aortic valve (TAV) (COA+TAV subgroup [n = 563 (65%)]). Aortic dimensions were assessed at baseline and at 3, 5, and 7 years. RESULTS: Compared with the no-SHD group, the COA+BAV subgroup had larger aortic root diameter (37 mm [Q1-Q3: 30-43 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001) and mid ascending aorta dimeter (34 mm [Q1-Q3: 29-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P = 0.008). Similarly, the BAV group had larger aortic root diameter (37 mm [Q1-Q3: 30-42 mm] vs 32 mm [Q1-Q3: 27-35 mm]; P < 0.001), and mid ascending aorta dimeter (35 mm [Q1-Q3: 30-40 mm] vs 28 mm [Q1-Q3: 24-31 mm]; P < 0.001). Compared with the COA+TAV subgroup, the COA+BAV subgroup and BAV group were associated with larger aortic root and mid ascending aorta diameter at baseline and follow-up. The risk of acute aortic complications was low in all groups. CONCLUSIONS: These findings suggest that BAV (and not COA) was associated with ascending thoracic aorta dimensions, and that patients with COA+TAV were not at a greater risk of developing ascending aortic aneurysms as compared with patients without SHD.
Asunto(s)
Aneurisma de la Aorta Ascendente , Aneurisma de la Aorta , Coartación Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Enfermedades de las Válvulas Cardíacas , Humanos , Válvula Aórtica/diagnóstico por imagen , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/epidemiología , Enfermedades de las Válvulas Cardíacas/complicaciones , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/epidemiología , Aneurisma de la Aorta/etiologíaAsunto(s)
Coartación Aórtica , Anomalías Cardiovasculares , Arteria Subclavia , Humanos , Arteria Subclavia/anomalías , Arteria Subclavia/diagnóstico por imagen , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/fisiopatología , Coartación Aórtica/complicaciones , Resultado del Tratamiento , Anomalías Cardiovasculares/diagnóstico por imagen , Aneurisma/diagnóstico por imagen , Masculino , Aortografía , FemeninoAsunto(s)
Coartación Aórtica , Estenosis de la Válvula Aórtica , Arteria Pulmonar , Función Ventricular Derecha , Humanos , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/fisiopatología , Coartación Aórtica/complicaciones , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/anomalías , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/fisiopatología , Estenosis de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/complicaciones , Masculino , Femenino , Hemodinámica , Circulación Pulmonar , Adulto , Valor Predictivo de las Pruebas , Adulto Joven , AdolescenteAsunto(s)
Coartación Aórtica , Aneurisma Intracraneal , Adulto , Humanos , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico por imagen , Coartación Aórtica/epidemiología , Aneurisma Intracraneal/complicaciones , Aneurisma Intracraneal/diagnóstico por imagen , Aneurisma Intracraneal/epidemiología , Incidencia , Aorta , Neuroimagen/efectos adversosRESUMEN
We previously reported short-term outcomes for stenting of aortic coarctation (CoA) (native or re-coarctation) with newer generation low-profile stents (Valeo, Formula, and Begraft stents) in children under 30 kg. We present here the medium-term outcomes of this procedure. Retrospective review of patients weighing under 30 kg who had percutaneous stent treatments for coarctation between 2012 and 2021 was performed. Clinical and procedural data were collected; 19 patients were included. The median age at the time of procedure was 5.1 [4.1-6.4] years and median weight 21.0 [17.3-22.3] kg. One patient had a history of re-coarctation. Thirteen (68%) patients were on anti-hypertensives pre-procedure. Different types of stents were used (14 Valeo™, 4 Formula® 535, 1 BeGraft), which can all be dilated to 18 mm or larger. One patient required a 9 F sheath, all others required a 7 F sheath. The narrowest diameter in the aorta increased from a median of 3.5 [3.0-4.5] to 9.4 [8.9-9.8] mm, p < 0.001; there was a reduction in the median pressure gradient across the coarctation from 35.0 [30.0-43.0] to 5.0 [0-10.0] mmHg, p < 0.001. There were no intra-procedural complications. Follow-up was for a median of 56.0 [13.0-65.0] months. Five (26%) of patients underwent re-intervention after a median time frame of 40.0 [39.5-52.0] months; four had balloon dilation, one had repeat stent implantation. Five (26%) patients were on anti-hypertensive agent(s) post-intervention. Our single centre experience demonstrates that percutaneous stenting for coarctation of aorta in children under 30 kg, with low-profile stents, had no significant complications during the median follow-up time of 56 months. This study demonstrated that the procedure is safe and effective for short and medium-term therapy in this group of patients with a 26% re-intervention rate. A quarter of patients remained on anti-hypertensive medication post stenting, emphasizing the importance of long-term follow-up.