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Transcatheter closure of perimembranous ventricular septal defect (PmVSD) is an established procedure. However, the occurrence of complete heart block limits its scope. The newer KONAR-MF™ occluder has specific design characteristics that may improve the safety of PmVSD closure. The objective of the study was to describe the efficacy and mid-term follow-up of transcatheter closure of PmVSD using KONAR-MF™. The study was conducted prospectively in 3 Indian centers (January 2018-December 2022). PmVSD closure was done by both antegrade and retrograde methods, and patients were followed up at 1, 3, 6, 12 months, and annually after that. 121 out of 123 patients were included with the following characteristics: median age 4.4 (0.18-40) years; weight 15 (2.1-88) kg; mean Qp/Qs ratio 1.87 ± 0.52 and pulmonary artery mean pressure: 22 ± 6.9 mmHg. The procedure was successful in all but 3; the device was removed due to significant residual shunt (n = 2) and new development of aortic regurgitation (AR) (≥ mild) in 1. The median defect size was 5.2 (2.5-12) mm. Device sizes from 6/4 to 14/12 were deployed (median fluoroscopy time 13.3 min; range 3.6-47.8). Shunt occlusion rates were 90%-Immediate, 95%-pre-discharge, and 97%-1 month, with no instances of complete heart block after the procedure and during follow-up. Six had new onset AR (mild: 2, trivial 4), and one had increased tricuspid regurgitation. All patients were well during follow-up (median: 20 months; range: 6-46). The new KONAR-MF™ occluder appears to be a promising and safe alternative for the closure of the PmVSD; further long-term follow is merited.
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To assess the feasibility and outcome of stent strut dilation after arterial duct stenting with associated branch pulmonary artery (BPA) stenosis. Stenting of arterial duct in infants with duct-dependent pulmonary circulation is technically challenging. The presence of BPA stenosis is a relative contraindication for stent implantation. Infants with duct-dependent pulmonary circulation and associated BPA stenosis were assessed either by transthoracic echocardiogram alone or additional computerized tomography angiogram when required. Following ductal stenting, the stent struts of the stenosed BPA were crossed with an additional 0.014â³ coronary guide wire and dilated using coronary balloons (2.0 or 2.5 mm in diameter). Seventeen (12 male) patients were considered for the procedure. The median age and weight were 27 days (range 2-94) and 2.6 kg (range 2.2-5), respectively. Fourteen patients (82.4%) underwent stent strut dilation after arterial duct stenting. Struts to left pulmonary artery was opened in 9 (64.3%) and right pulmonary artery in 5 (35.7%). The mean systemic oxygen saturation increased from 66.23 ± 8.9% at baseline to 86 ± 2.2% immediately after the stent deployment and final saturations after stent strut dilation were 89.29 ± 4.3%. Angiographic pulmonary flow improved in all cases. Stent strut dilation could not be done in 3 patients due to unfavorable anatomy. One patient had acute stent thrombosis and died in the hospital. Two others died during follow-up, during an acute febrile illness and gastroenteritis. All survivors underwent cardiac surgery and were on regular follow-up. Strut dilation of BPA stenosis is feasible to augment pulmonary blood flow, following arterial duct stenting. This procedure may be useful in selected patients with BPA stenosis to have uniform growth of pulmonary arteries.
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The prevalence of coronavirus disease-2019 (COVID-19) is about 2% in children. Varied manifestations including pneumonia, myocarditis, and multisystem inflammatory syndrome, namely is commonly seen in children. Aortic pseudoaneurysm due to COVID-19 is rare and carries a poor prognosis. We came across a 5-year-girl who had presented with heart failure due to a large ascending aortic pseudoaneurysm associated with COVID-19 during the first wave of the pandemic. The patient underwent successful surgical repair.
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Aicardi-Goutières syndrome (AGS) induces innate immune activation. It can present with cerebral calcifications and hepatosplenomegaly mimicking congenital infections. The present case report discusses the diagnosis and treatment of a case of fetal cardiomyopathy whose postnatal symptoms resembled TORCH (toxoplasmosis, other agents, rubella, cytomegalovirus, herpes and syphilis) infection. The mother had a history of two lost pregnancies due to fetal cardiomyopathy and the same was identified in the current pregnancy. At 34 weeks of gestation, the mother delivered a late preterm male neonate due to intrauterine growth restriction weighing 1590 g with respiratory distress and cardiomyopathy at birth. The neonate had cerebral calcifications, hepatosplenomegaly and thrombocytopenia. As the infant's TORCH IgM titre was negative, pseudo-TORCH syndrome similar to AGS was suspected. Clinical exome sequencing of the parents and fetus identified no genes for hydrops fetalis or fetal cardiomyopathy; however, the AGS TREX1 gene was identified in the neonate, while additional symptoms resembled TORCH infection. The neonate was discharged and has shown improvement with oral baricitinib treatment for the last 9 months.
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Enfermedades Autoinmunes del Sistema Nervioso , Infecciones por Herpesviridae , Rubéola (Sarampión Alemán) , Toxoplasmosis , Embarazo , Recién Nacido , Lactante , Femenino , Humanos , Masculino , Rubéola (Sarampión Alemán)/diagnóstico , Toxoplasmosis/diagnósticoRESUMEN
Percutaneous transcatheter closure of fossa ovalis atrial septal defect (FOASD) is an established procedure. When femoral venous approach is unfeasible due to interrupted inferior vena cava (IVC) or occluded femoral veins, other interventional methods through transjugular, transhepatic approach, or surgical closure can be performed. We report two cases of transhepatic device closure of FOASD associated with IVC interruption successfully performed without any complications.
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Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5-21) and 19.5 kg (4.2-73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days-4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.
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BACKGROUND: Outcome data of children with heart disease who acquired COVID-19 infection are limited. AIMS: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. MATERIALS AND METHODS: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. RESULTS: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046). CONCLUSIONS: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.
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BACKGROUND: Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth. OBJECTIVES: The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome. METHODS: This is a retrospective observational study between January 2014 and December 2019. Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. Postnatal evaluation by transthoracic echocardiography and computerized tomography angiogram was performed. Surgical repair was done as per standard indications. RESULTS: A total of 35 cases of fetal VRs (median gestational age: 24 weeks [range: 19-35]) were diagnosed during the study period. There were four dichorionic diamniotic twin gestation pregnancies. The right aortic arch (RAA) with anomalous left subclavian artery (ALSA) was suspected in 31 fetuses, double aortic arch (DAA) in 3, and circumflex aorta in 1. Twenty-six (74%) patients had successful deliveries. One patient had a spontaneous miscarriage, 2 underwent termination, and 6 were lost to follow-up. Postnatal assessment showed RAA with ALSA in 18, DAA in 5, circumflex aorta in 2, and no abnormality in 1. Twenty-two (86%) were operated (RAA with ALSA: 17, DAA: 4, and circumflex aorta: 1) and four were waiting for surgery. Two patients died due to prematurity-related complications. All survivors are symptom free during follow-up (median: 2.24; range: 0.2-5.6 years). CONCLUSIONS: Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs.
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An 8-day-old neonate was presented with severe respiratory distress and diagnosed as primary pulmonary hypertension of the newborn on functional echocardiogram. Evaluation showed bounding pulse, enlarged umbilical cord, and bruit over the periumbilical region. Transthoracic echocardiography and CT angiogram showed a large fistulous communication between the umbilical vein and artery suggestive of congenital umbilical arteriovenous malformation leading to high-output cardiac failure and pulmonary artery hypertension. The patient was stabilised with medications and ventilation. Transcatheter closure of communication was done using coils, vascular plug, and KONAR-MFTM device. The patient improved from heart failure soon after the procedure and thriving normally at 6 months of follow-up.
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Malformaciones Arteriovenosas , Insuficiencia Cardíaca , Malformaciones Arteriovenosas/diagnóstico por imagen , Malformaciones Arteriovenosas/terapia , Ecocardiografía , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/terapia , Humanos , Recién Nacido , Venas UmbilicalesRESUMEN
Shock is a state of circulatory dysfunction and its diagnosis is complex in neonates. Hemodynamic assessment using echocardiography has potential to guide better management regimes in neonates with shock. Objective of this study is to analyze changes in the echocardiographic parameters in preterm neonates with shock at presentation and after resolution. In this prospective pragmatic Cohort study, eligible neonates with shock were monitored for changes in echocardiographic parameters at onset of shock and after resolution of shock. Paired data analysis was done for observed changes in the parameters. Based on initial clinical parameters and echocardiographic parameters, infants were assigned into different types of shock. Data of 37 infants were analyzed for baseline clinical and echocardiographic parameters, and data of 31 infants were analyzed for the changes in the observed parameters after shock resolution. Statistically significant changes were observed in inferior vena cava collapsibility index (ICI), left ventricular end diastolic volume (LVEDV), isovolemic ventricular relaxation time (IVRT), left and right ventricular stroke volume, and ejection fraction (EF). There was no agreement between clinical and echocardiographic definitions of shock.Conclusion: We noticed shock has overlapping pathophysiologic features. Our study highlights the importance of baseline documentation of echocardiographic parameters of all infants who are at risk of shock and repeat echocardiography at onset of shock to observe the changes in ICI, LVEDV, IVRT, stroke volume, and EF. This would guide pathophysiological management of shock in neonates. What is Known: ⢠In neonates pathophysiology of shock is overlapping. ⢠Echocardiography can help in better understanding and management of shock. What is New: ⢠Study gives median changes in major echocardiographic parameters in neonatal shock. ⢠These changes can guide for selection of volume and inotropes in management.
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Ecocardiografía , Hemodinámica , Choque , Estudios de Cohortes , Humanos , Lactante , Recién Nacido , Estudios Prospectivos , Choque/diagnóstico , Volumen SistólicoRESUMEN
We report two cases of agenesis of the right pulmonary veins (PVs) associated with sequestration of the right lung with systemic to pulmonary collateral. Both the children were referred for evaluation of recurrent lower respiratory tract infections. Transthoracic echocardiographic evaluation showed reversal of flow in the right pulmonary artery (PA) and absent right PVs. Cardiac catheterization confirmed the diagnosis of agenesis of the right PVs. Transcatheter closure of collaterals was performed in view of significant shunt from systemic to PA. Both the patients are asymptomatic on follow-up.
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BACKGROUND: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. OBJECTIVE: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation. METHODS: This is a single-center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8. days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with variable penetrating characteristics. RESULTS: The procedure was successful in 16 of twenty patients using CTO wires: Shinobi in nine, Miracle in four, CROSS-IT in two, and Conquest Pro in one. Two patients had perforation of right ventricular outflow tract (RVOT). Pericardiocentesis was required in one patient to relieve cardiac tamponade. Later, the same patient underwent successful hybrid pulmonary valvotomy. The other patient underwent ductus arteriosus (DA) stenting. Balloon atrial septostomy was needed in three cases with systemic venous congestion. Desaturation was persistent in five cases necessitating DA or RVOT stenting to augment pulmonary blood flow. There were two early and two late deaths. The mean follow-up was 22.66 ± 16 months. Three patients underwent one and half ventricle repair and one Blalock-Taussig shunt during follow-up. CONCLUSION: Perforation of the pulmonary valve can be done successfully using CTO wires in selected cases of pulmonary atresia with intact ventricular septum.
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A 3-day-old neonate presented with features suggestive of coarctation of aorta. Echocardiography showed a large organized thrombus in the transverse arch causing obstruction to theaortic arch and carotids with partial recanalization. Patient underwent surgical thromboendarterectomy with arch reconstruction. The evaluation did not reveal any hematological abnormalities and suspected to be due to fetal thromboembolism. Patient improved well, and no neurological deficits were observed during follow-up.
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We report the successful re-screwing of the embolized duct occluder (DO) in three children for retrieval and to attempt redeployment. The DO was embolized into descending aorta immediately after the deployment in one child and within 24 h after the procedure in two further patients. The DO was re-screwed back by the DO delivery cable, using "sheath in sheath" in all three cases; however, successful retrieval could be done only in two. Repositioning in the patent ductus arteriosus (PDA) was done using the same device in those two children and surgical removal was needed in third child with perimembranous ventricular septal defect.
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We report on emergency percutaneous renal artery stenting in two children who developed acute anuria due to bilateral renal artery stenosis and near-total occlusion. In both children, urine output could be reestablished following the procedure. One patient died from persistent severe cardiac failure. Emergency stenting is feasible, and may buy time for future procedures.
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Lesión Renal Aguda/terapia , Angioplastia de Balón/instrumentación , Obstrucción de la Arteria Renal/terapia , Stents , Arteritis de Takayasu/complicaciones , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/etiología , Adolescente , Angioplastia/métodos , Angioplastia de Balón/métodos , Anuria/diagnóstico , Anuria/etiología , Anuria/terapia , Aorta Abdominal/diagnóstico por imagen , Aorta Abdominal/fisiopatología , Aorta Torácica/diagnóstico por imagen , Aorta Torácica/fisiopatología , Aortografía/métodos , Niño , Urgencias Médicas , Estudios de Seguimiento , Humanos , Masculino , Obstrucción de la Arteria Renal/diagnóstico por imagen , Obstrucción de la Arteria Renal/etiología , Medición de Riesgo , Arteritis de Takayasu/diagnóstico por imagen , Resultado del TratamientoRESUMEN
BACKGROUND: Surgical or transcatheter closure of muscular ventricular septal defects (mVSDs) in young children may be technically challenging and associated with significant complications. OBJECTIVE: To assess the feasibility of trans-septal antegrade closure of mVSD in a selected subset of young children. METHODS: This is a prospective study from a single centre from July 2011 to March 2013. Nine infants and children with single or multiple mVSDs were included in the study. The median age and weight were 6 months (range 4-18 months) and 4.5 kg (range 3.8-6.2 kg), respectively. Trans-femoral trans-septal antegrade technique was used in eight children. One child was excluded from the study because of abnormally tortuous anatomy of both the femoral veins and subsequently underwent VSD device closure by the trans-jugular approach. The follow-up evaluation included chest X-ray, ECG, and echocardiogram at 1 month, 3 months, 6 months, and 1 year. RESULTS: The defects were closed successfully in all eight patients using Amplatzer mVSD device in 5 and Amplatzer Duct Occluder II in 3. Moderate mitral regurgitation due to entrapment of the anterior mitral leaflet occurred in one patient with a posteriorly located mVSD, necessitating removal of the device, and surgical closure of the mVSD. The small additional residual mVSD in one other patient closed spontaneously during the follow-up. CONCLUSIONS: Transcatheter trans-septal antegrade closure of mVSD in young children is technically feasible and merits further consideration. Symptomatic relief in multiple mVSD can be achieved after closing larger defects.
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Cateterismo Cardíaco , Defectos del Tabique Interventricular/terapia , Factores de Edad , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Cateterismo Cardíaco/métodos , Ecocardiografía Doppler en Color , Electrocardiografía , Estudios de Factibilidad , Femenino , Defectos del Tabique Interventricular/diagnóstico , Humanos , India , Lactante , Masculino , Insuficiencia de la Válvula Mitral/etiología , Estudios Prospectivos , Diseño de Prótesis , Dispositivo Oclusor Septal , Factores de Tiempo , Resultado del TratamientoRESUMEN
We report a successful perventricular closure of an apical muscular ventricular septal defect (mVSD) by a modified technique. An eight-month-old infant, weighing 6.5 kilograms, presented with refractory heart failure. The transthoracic echocardiogram showed multiple apical mVSDs with the largest one measuring 10 mm. perventricular device closure using a 12 mm Amplatzer mVSD occluder was planned. The left ventricular disk was positioned approximating the interventricular septum; however, the right ventricular (RV) disk was deployed on the free wall of the RV due to an absent apical muscular septum and a small cavity at the apex. The RV disk of the device was covered using an autologous pericardium. His heart failure improved during follow-up.