Truncus arteriosus communis associated with underdevelopment of the aortic arch.

Two cases are reported of truncus arteriosus communis arising entirely from the right ventricle and associated with interruption in one case and tubular hypoplasia of the aortic arch in the other. A haemodynamic hypothesis of preferential blood flow is proposed to explain this rare association.     

Truncus arteriosus communis associated with underdevelopment of the aortic arch.

Two cases are reported of truncus arteriosus communis arising entirely from the right ventricle and associated with interruption in one case and tubular hypoplasia of the aortic arch in the other. A haemodynamic hypothesis of preferential blood flow is proposed to explain this rare association.     

Rare variant of truncus arteriosus with intact ventricular septum and hypoplastic right ventricle.

A three week old girl was admitted to hospital with severe congestive heart failure and cyanosis. Cross sectional and Doppler echocardiography and cardiac catheterisation showed a unique variant of truncus arteriosus with an intact ventricular septum. The trunk rose only from the left ventricle and was associated with a hypoplastic right ventricle with sinusoids to the right coronary artery.     

Rare variant of truncus arteriosus with intact ventricular septum and hypoplastic right ventricle

A three week old girl was admitted to hospital with severe congestive heart failure and cyanosis. Cross sectional and Doppler echocardiography and cardiac catheterisation showed a unique variant of truncus arteriosus with an intact ventricular septum. The trunk rose only from the left ventricle and was associated with a hypoplastic right ventricle with sinusoids to the right coronary artery.     

Rare variant of truncus arteriosus with intact ventricular septum and hypoplastic right ventricle

A three week old girl was admitted to hospital with severe congestive heart failure and cyanosis. Cross sectional and Doppler echocardiography and cardiac catheterisation showed a unique variant of truncus arteriosus with an intact ventricular septum. The trunk rose only from the left ventricle and was associated with a hypoplastic right ventricle with sinusoids to the right coronary artery.     

Large ductus arteriosus and intact ventricular septum associated with congenital absence of the pulmonary valve.

Clinical, haemodynamic, angiocardiographic, and necropsy findings are reported in a newborn infant who presented with typical findings of the severe form of the syndrome of absent pulmonary valve. In addition to the usual findings of aneurysmal dilatation of the pulmonary arteries, pulmonary insufficiency, and stenosis, this patient had an intact ventricular septum and large ductus arteriosus. The former is a rare finding and the latter is not known to have been reported in a patient with the severe form of this syndrome.     

Truncus arteriosus communis,an unusual anatomical variant of type I

We present a case of type I truncus arteriosus communis characterized by the horizontal origin of the main pulmonary artery from the common trunk and by a winding course up to a total length of 11.5 cm to the point of bifurcation of the pulmonary artery. Other than cyanosis the patient had a normal clinical history, which was followed for 20 years. The length of the main pulmonary artery could be related to the lower site of the incomplete septation of the common trunk, and it seems that hemodynamic factors contributed to this unusual configuration.     

Pulmonary atresia with intact ventricular septum

Opinion statement The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with and without conventional surgical treatment. Because of this reason, a comprehensive program of medical, transcatheter, and surgical treatment is necessary to improve the long-term outlook of these infants. Algorithms of management plans should be developed based on the presence of right ventricular-dependent coronary circulation as well as size and morphology of the right ventricle. In a tripartite or bipartite right ventricle, transcatheter radiofrequency perforation is preferable. Alternatively, surgical valvotomy may be performed. Augmentation of pulmonary blood flow by prolonged infusion of prostaglandin E₁, stenting the ductus, or a surgical modified Blalock-Taussig shunt may be necessary in some of these patients. In patients with a unipartite or very small right ventricle or a right ventricular-dependent coronary circulation (Tricuspid valve Z score < -2.5), augmentation of pulmonary flow along with atrial septostomy should be undertaken. Follow-up studies to determine the feasibility of biventricular repair should be undertaken and, if feasible, surgical or transcatheter methods may be used to achieve the goals. If not suitable for biventricular repair, one-ventricle (Fontan) or one and one-half ventricular repair should be considered. Comprehensive and well-planned treatment algorithms may help improve survival rate.     

Truncus arteriosus communis: truncal valve anomalies associated with small conal or truncal septal defects.

Selected specimens of truncus arteriosus communis illustrate a spectrum of anomalous truncoconal septation in which the conal and truncal septal defects are so closely approximated to the truncal valve that portions of the valve tissue extend along the free margins of the adjacent ventricular or truncal septum.     

Criss-cross heart with intact ventricular septum

We report a case of a criss-cross heart in a 6-month-old male child with echocardiographic and angiocardiographic findings of concordant atrioventricular and discordant ventriculoarterial connexions and an intact ventricular septum. This entity has been described with a ventricular septal defect as part of its own anatomic spectrum. The concept of the criss-cross heart, however, is best restricted to a form of spatial atrioventricular arrangement in which the atrioventricular connexions are criss-crossed.     

Truncus arteriosus communis associated with interrupted aortic arch: a report on two uncommon cases.

The paper presents two infants with the A-4 type of truncus arteriosus communis (according to Van Praagh's classification). One patient who survived a surgical procedure demonstrated a rare variant of aortic arch interruption to the left off the left subclavian artery (type A according to Celoria and Patton), whereas the second presented an uncommon anomaly in which the right subclavian artery originated from the descending aorta with associated severe truncal valve incompetency. The authors describe the clinical picture along with the surgical treatment of the first infant who being six days old was subjected to a correction employing the wide patent ductus arteriosus to reconstruct the aortic arch, following the method described by Gomes and McGoon. Subsequently an aortic homograft was implanted in order to connect the right ventricle and the pulmonary artery.     

Large ductus arteriosus and intact ventricular septum associated with congenital absence of the pulmonary valve

Clinical, haemodynamic, angiocardiographic, and necropsy findings are reported in a newborn infant who presented with typical findings of the severe form of the syndrome of absent pulmonary valve. In addition to the usual findings of aneurysmal dilatation of the pulmonary arteries, pulmonary insufficiency, and stenosis, this patient had an intact ventricular septum and large ductus arteriosus. The former is a rare finding and the latter is not known to have been reported in a patient with the severe form of this syndrome.     

Critical pulmonary valve stenosis with intact ventricular septum.

This study presents our experience in the surgical management of the newborn with critical pulmonary stenosis and intact ventricular septum. During the last decade we have seen 11 such patients, with a mean age of 2.3 days (maximum 6 days) and a mean weight of 3.6 kilograms (maximum 4.5 kilograms). The primary operation was a valvotomy in 7 patients (one of whom also had a systemic-pulmonary shunt), a commissurotomy with cardiopulmonary bypass in 3 patients, and a shunt alone in 1 patient. One infant died on the second postoperative day and another died following reoperation at 4 months. The other 9 patients were all alive and progressing satisfactorily when last seen. Five of these patients were beyond their third postoperative year and the longest survivor was at 7.9 years. Four of the long-term survivors had undergone reoperation. Our choice of operation remains transventricular valvotomy, although an open procedure utilizing cardiopulmonary bypass presents an attractive alternative. A systemic-pulmonary shunt as an addition to either of these operations is unnecessary. The perioperative use of prostaglandin E brings about substantial improvement in the early results.