Search Results (5)

Sinus of Valsalva aneurysms (SVAs) are infrequently seen in the pediatric population. When these aneurysms rupture, a significant hemodynamic burden is placed on the heart and increases the likelihood of cardiac failure. Here, we report a case of a ruptured SVA into the ventricular myocardium in a fetus with a form of double-inlet left ventricle. To the best of our knowledge, this has not previously been described. Full article

Double-inlet left ventricle (DILV) is most frequent among univentricular atrioventricular connections. In DILV, there is a single functioning ventricle, most commonly with left ventricular structure. This chamber receives both atrioventricular valves and is connected to an outlet chamber with morphologic features of the right ventricle. The great vessels are often transposed, and pulmonary stenosis is seen in two-thirds of patients. The anatomy and pathophysiology can be defined by echo-Doppler studies with a rare need for other imaging studies. The management is mostly related to the nature of associated heart defects and the degree of pathophysiological abnormality. When the infants present initially, treatment to address the hemodynamic issues is undertaken. Subsequently, these babies need staged total cavo-pulmonary connection, i.e., the Fontan procedure which is undertaken in three stages; these stages are described in this review. The existence of inter-stage mortality and post-Fontan complications is recognized and was reviewed. The paper concludes that DILV can be successfully diagnosed with echo-Doppler studies and this heart anomaly can be effectively treated with the currently prevailing medical, catheter interventional, and surgical treatment practices. Full article

Common arterial trunk (CAT) is a rare congenital heart disease that is commonly included into the spectrum of conotruncal heart defects. CAT is rarely associated with functionally univentricular hearts, and only few cases have been described so far. Here, we describe the anatomical characteristics of CAT associated with a univentricular heart diagnosed in children and fetuses referred to our institution, and we completed the anatomical description of this rare condition through an extensive review of the literature. The complete cohort ultimately gathered 32 cases described in the literature completed by seven cases from our unit (seven fetuses and one child). Four types of univentricular hearts associated with CAT were observed: tricuspid atresia or hypoplastic right ventricle in 16 cases, mitral atresia or hypoplastic left ventricle in 12 cases, double-inlet left ventricle in 2 cases, and unbalanced atrioventricular septal defect in 9 cases. Our study questions the diagnosis of CAT as the exclusive consequence of an anomaly of the wedging process, following the convergence between the embryonic atrioventricular canal and the common outflow tract. We confirm that some forms of CAT can be considered to be due to an arrest of cardiac development at the stages preceding the convergence. Full article

In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation. Full article

In this review management of the most common cyanotic congenital heart defects (CHDs) was discussed; the management of acyanotic CHD was reviewed in Part I of this series. While the need for intervention in acyanotic CHD is by and large determined by the severity of the lesion, most cyanotic CHDs require intervention, mostly by surgery. Different types of tetralogy of Fallot require different types of total surgical corrective procedures, and some may require initial palliation, mainly by modified Blalock–Taussig shunts. Babies with transposition of the great arteries with an intact ventricular septum as well as those with ventricular septal defects (VSD) need an arterial switch (Jatene) procedure while those with both VSD and pulmonary stenosis should be addressed by Rastelli procedure. These procedures may need to be preceded by prostaglandin infusion and/or balloon atrial septostomy in some babies. Infants with tricuspid atresia require initial palliation either with a modified Blalock–Taussig shunt or banding of the pulmonary artery and subsequent staged Fontan (bidirectional Glenn and fenestrated Fontan with extra-cardiac conduit). Neonates with total anomalous pulmonary venous connection are managed by anastomosis of the common pulmonary vein with the left atrium either electively in non-obstructed types or as an emergency procedure in the obstructed types. Babies with truncus arteriosus are treated by surgical closure of VSD along with right ventricle to pulmonary artery conduit. The other defects, namely, hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, double-outlet right ventricle, double-inlet left ventricle and univentricular hearts largely require multistage surgical correction. The currently existing medical, trans-catheter and surgical techniques to manage cyanotic CHD are safe and effective and can be performed at a relatively low risk. Full article