Treatment-refractory heart failure as a manifestation of aortic arch atresia / Falla cardiaca refractaria a tratamiento como manifestación de atresia de arco aórtico

Abstract Background: Distal segment atresia (isthmus) is an extremely rare anatomical variant of obstructive aortic arch anomalies. Case report: We present the case of a newborn who, at 48 hours of life, presented a clinical picture of heart failure. The initial echocardiogram showed a congenital interrupted aortic arch type A, patent ductus arteriosus, and ventricular septal defect. Prostaglandins were initially indicated. Subsequently, a second echocardiogram showed the absence of ductus arteriosus; the CT angiography study confirmed this finding and revealed blood flow to the descending aorta through small intercostal blood vessels. The possibility of atresia of the distal segment (isthmus) of the aortic arch was considered and confirmed at the time of surgery. Conclusions: Aortic atresia should be considered a diagnostic possibility in the presence of type A interrupted aortic arch since the hemodynamic behavior between them is similar. Surgical medical treatment should be individualized since this condition is frequently an emergency in the neonatal period. However, this is not always the case, as other cases have been reported in schoolchildren and adults.

Resumen Introducción: La atresia de segmento distal (istmo) de arco aórtico es una variante anatómica extremadamente rara de las anomalías obstructivas del arco aórtico. Caso clínico: Se presenta el caso de un recién nacido que a las 48 horas de vida presentó un cuadro clínico de insuficiencia cardiaca. El estudio de ecocardiograma inicial mostró una anomalía congénita de interrupción de arco aórtico tipo A, conducto arterioso y comunicación interventricular. De inicio se indicaron prostaglandinas. Posteriormente, el segundo ecocardiograma mostró la ausencia del conducto arterioso; el estudio de angiotomografía confirmó este hallazgo y también reveló flujo sanguíneo hacia aorta descendente a través de pequeños vasos sanguíneos intercostales. Se consideró la posibilidad de atresia del segmento distal (istmo) de arco aórtico y se confirmó al momento del acto quirúrgico. Conclusiones: La atresia aórtica debe ser considerada como posibilidad diagnóstica en presencia de interrupción de arco aórtico tipo A, ya que el comportamiento hemodinámico entre ellos es similar. El tratamiento médico quirúrgico debe individualizarse, ya que es frecuente que sea una urgencia en el periodo neonatal. Sin embargo, no sucede así siempre, ya que se han reportado casos en escolares y adultos.

Yasui procedure for surgical repair of interruption of aortic arch and ventricular septal defect associated with aortic stenosis or aortic atresia / 中华胸心血管外科杂志

Objective:To study the surgical results of Yasui procedure for interruption of aortic arch and ventricular septal defect(IAA/VSD) associated with aortic stenosis(AS) or aortic atresia(AA).Methods:A retrospective study was performed on all 4 children with IAA/VSD/AS(AA) that underwent a Yasui procedure from Dec 2014 to Dec 2019. Three patients had AS, and 1 patient had AA. The age was from 10 days to 25 months and the weight was from 2.7 kg to 10 kg. The type of IAA was type A in 1 and type B in 3. The diameter and Z value of the aortic annulus were 1.9-4.3 mm and -6.7--3.6. The diameter and Z value of the ascending aorta were 2.6-5.8 mm and -5.4--2.6. The mean Z value of LVEDD and LVEDS was 3.6±2.6 and 3.6±2.9 resparately.Results:All 4 patients survived after the Yasui procedure. Three patients underwent primary repair and 1 patient underwent staged repair. CPB time was 128-283 min and aortic cross-clamping time was 98-171 min. Ventilation time was 93-296 h, and ICU and hospital length of stay was 7-25 days and 18-39 days. Follow-up was complete in all patients from 8 to 92 months, and there was no late death. All the patients were in good biventricular function with NYHA grade Ⅰ in 3 and NYHA grade Ⅱ in 2 patients. No residual left ventricular outflow tract obstruction was detected.Conclusion:The Yasui procedure can be an option for patients with IAA/VSD/AS(AA) and good developed left ventricle and can achieve good early surgical results.

Prenatal ultrasound diagnosis of fetal aortic atresia / 中华超声影像学杂志

Objective To investigate the clinical value of prenatal ultrasonography in diagnosing fetal aortic atresia.Methods Ultrasound images of 9 cases with fetal aortic atresia confirmed by autopsy were retrospectively analysed and summarized.Results Fetal echocardiography showed ascending aorta and aortic arch were thin in seven cases and unclear in two cases.Color Doppler flow imaging showed fetal aorta no blood connection with left ventricle and reverse blood flow in aortic arch among all 9 cases.There were 6 cases associated with hypoplastic left heart syndrome,two cases associated with ventricular septal defect and well-developed left ventricle,one case complicated with complete atrioventricular septal defect.Nine cases were detected at average gestational age (26.0 ± 2.6) weeks,minimum gestational age was only 16 weeks pregnant.Conclusions Prenatal ultrasound diagnosis of fetal aortic atresia has important clinical value.Ascending aorta and aortic arch are thin or unclear,no blood flow connection between fetal aorta and left ventricle and reverse blood flow in aortic arch are major diagnostic evidences.Color Doppler flow imaging is helpful for early diagnosis.

The Norwood-Rastelli Procedure for Left Ventricular Outflow Tarct Obstruction with a Ventricular Septal Defect: Three case report / 대한흉부외과학회지

Between 2001 and 2006, 3 neonates that had multilevel left ventricular outflow tract obstruction and a ventricular septal defect underwent the Norwood-Rastelli procedure. The body weights ranged from 2.9 to 3.1 kg. The patients had a near normal sized mitral valve and left ventricle. We simultaneously performed a modified Norwood procedure with native tissues-to-tissue anastomosis without circulatory arrest, and a Rastelli type procedure using a non-valved conduit from the right ventricle to the pulmonary artery and intracardiac patch baffling from the left ventricle to the pulmonary valve via the ventricular septal defect. The postoperative courses were uneventful. During follow-up, there was one late mortality caused by a cardiac catheterization related complication at 7 months after surgery. One patient required a Rastelli conduit change. Two patients are doing well during a follow-up period of 1 and 5 years, respectively.

Cord-like Atresia of the Abdominal Aorta Due to Takayasu Arteritis in Middle Aged Woman: A case Report / 대한흉부외과학회지

Takayasu arteritis is a chronic non-specific inflammatory arteriopathy that involves primarily the first branches of aortic arch but may also affect the aorta and any of its primary branches. A characteristic trait of the disease is that most of the patients are young females of mainly Asian and South American origin. Recently, we experienced a rare case of Takayasu arteritis which showed a cord-like atresia of abdominal aorta just below renal arteries in 52-year-old woman. We performed a side to side bypass graft from descending thoracic aorta to abdominal aorta just above the aortic bifurcation with a 18mm PTFE(polytetrafluoroethylene) vascular graft. The postoperative course was uneventful.

A Case of Dandy-Walker Syndrome with Complex Cardiac Anomaly

The Dandy-Walker syndrome is a developmental disorder of the brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We experinced a case of Dandy-Walker syndrome with complex cardiac anomaly in female newborn who presented with apnea at birth. Physical examination showed coloboma on left eye, low estting malformed ear, and high arched palate. Autopsy revealed cystic dilatation of the fourth ventricle with secondary loss of cerebellar vermis. The floor of the fourth ventricle is exposed through this defect. Heart showed aortic atresia and univentricular heart. The case is reported with the review of the literatures.

Síndrome de hipoplasia do coraçäo esquerdo: avaliaçäo clínica de 40 casos / Hypoplastic left heart syndrome. Clinical evaluation of 40 patients

Estudar a síndrome de hipoplasia do coraçäo esquerdo (SHCE), enfatizando aspectos clínicos e conduta terapêutica. Quarenta pacientes, 24 do sexo masculino, com idades entre um e 120 (média seis) dias. O diagnóstico foi baseado no quadro clínico, eletrocardiograma. Oito pacientes foram submetidos a caterismo cardíaco. Em 21 casos houve confirmaçäo por estudo necroscópico. Vinte e dois (55%) pacientes evoluiram para o óbito apesar das medidas clínicas instituídas. Em 16 (40%) realizou-se cirurgia paliativa, técnica de Norwood em 14 e bandagem do tronco pulmonar com interposiçäo de tubo de dacron entre este e o tronco braquiocefálico em dois. Dos pacientes submetidos a cirurgia de Norwood, três tiveram resultados imediatos favoráveis. Os pacientes submetidos ao outro procedimento citado, faleceram nos 12§ e 30§ dias de pós-operatório, em conseqüência de infecçäo. A SHCE é cardiopatia grave, com insuficiência cardíaca de manifestaçäo precoce (geralmente na primeira semana de vida) e de evoluçäo inexorável para o óbito quando se utilizam apenas as medidas clínicas habituais. O sucesso das técnicas cirúrgicas paliativas depende basicamente do diagnóstico precoce e do manuseio clínico adequado

Purpose: To study the hypoplastic left heart syndrome (HLHS) emphasizing clinical aspects and therapeutic measures. Patients and Methods: Forty patients, 24 male, ages ranging between one to 120 days (mean six days). Diagnosis was established by clinical elements, eletrocardiogram, chest X-ray and Doppler echocardiogram. Eight patients underwent cardiac catheterization. In 21 patients anatomical diagnosis was confirmed by necropsy. Results: Twenty-two patients (55%) died, in spite of all possible clinical measures. In 16 (40% ) palliative surgery was performed, Norwood technique in 14 and pulmonary artery banding with Dacron graft interposition between pulmonary trunk and brachiocephalic trunk in two. Immediate postoperative results were favorable in three patients (Norwood technique). Both patients underwent the other described technique died at 12th and 30th postoperative day due to infection. Conclusion: HLHS is a severe cardiac anomaly with a fast downhill evolution mostly at first week of life. The good results depend basically of early diagnosis as well as of an adequate clinical management for an appropriate time in indication of the palliative surgical technique.