Anomalous origin of both pulmonary arteries from the ascending aorta with a nonbranching main pulmonary artery arising from the right ventricle

Summary A 3-month-old boy, mildly cyanosed and tachypneic, was found by cineangiography to have a nonbranching main pulmonary artery arising from the right ventricle and connecting to the descending aorta via a large persistent ductus arteriosus. The left and right pulmonary arteries arose as a common trunk, before branching, from the ascending aorta. Complete surgical correction was performed at 7-months of age. Subsequent cardiac catheterization has demonstrated normal pressures and blood oxygen saturation in all right-heart chambers and the great arteries.     

Right aortic arch with isolation of the left subclavian artery

A 5-year-old boy with cyanotic heart disease and weak pulses in the left arm is described. Cardiac catheterization and cineangiography confirmed the diagnosis of tetralogy of Fallot and right aortic arch with isolation of the left subclavian artery. In addition to the aortogram and right ventricular cineangiography, pulmonary angiography was performed, demonstrating that blood did not reach the left subclavian artery through a left ductus arteriosus.     

Single Doppler Detection of Left to Right Shunt through the Ductus Arteriosus

ABSTRACT. Forty-two infants and children were examined with unguided continuous and pulsed Doppler echocardiography before and/or after surgical closure of ductus arteriosus or in connection with cardiac catheterization. Presence or absence of diastolic reverse flow in the main pulmonary artery was evaluated for sensitivity and specificity to detect left to right ductus shunt. Diastolic reverse flow was detected in 15 of 16 patients with such a shunt and in 3 of 38 patients without a left to right ductus shunt. This corresponds to 94% sensitivity and 92% specifcity. It is concluded that the accuracy of a single Doppler system in diagnosing patent ductus arteriosus is comparable to the results obtained with a Doppler interfaced to M-mode or cross-sectional echocardiography.     

Imperforate tricuspid valve with dysplasia of the right ventricular myocardium,pulmonary valve,and coronary artery: A clinicopathological study of nine cases

Summary Nine cases of imperforate tricuspid valve associated with dysplasia of the right ventricular myocardium, pulmonary valve, and right coronary artery are described. The mean frontal QRS axis of the electrocardiograms did not indicate left axis deviation in seven of the nine. Two-dimensional echocardiograms showed an imperforate tricuspid valve, normally aligned atrioventricular septum, and an irregular-shaped right ventricular cavity with a thin wall. In three cases with absence of the pulmonary valve, pulsed Doppler echocardiograms of the right ventricular outflow tract revealed antegrade flow during systole and retrograde flow during diastole, and selective angiography through a persistent ductus arteriosus showed retrograde filling of the blind-ended right ventricular cavity via the main pulmonary artery. Necropsy examinations showed an imperforate tricuspid valve with a thin-walled aneurysmal right ventricle in all six cases examined. Pulmonary atresia was observed in two cases, and the absence of the pulmonary valve in four. The right coronary artery was hypoplastic in four cases and absent in two. A shunt operation should be carried out in early infancy to prevent hypoxia after closure of the ductus arteriosus.     

Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced,reversible, left-sided pulmonary hypertension

Summary An infant with scimitar syndrome, absent right pulmonary artery, and systemic blood supply to the right lung presented in severe cardiac failure. Cardiac catheterization revealed suprasystemic pressure of the left pulmonary artery and a high pulmonary vascular resistance. Right-sided pneumonectomy abolished cardiac failure and normalized both pulmonary artery pressure and resistance. Pure volume load affecting one lung—as in this case through absence of the right pulmonary artery plus additional left-to-right shunt from a systemic collateral—can lead to pulmonary hypertension. Early operative intervention can reverse this process and prevent pulmonary vascular disease.     

Large patent ductus arteriosus and interventricular communication associated with congenital absence of the pulmonary valve

A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.     

Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery

A case of tetralogy of Fallot associated with anomalous origin of the right pulmonary artery from the ascending aorta and hypoplastic left pulmonary artery was successfully repaired. Following the simultaneous procedure with banding of the right pulmonary artery and replacement of modified Blalock-Taussig shunt to the left pulmonary artery at 34 days of age, the intracardiac repair was performed successfully at 1 year and 10 months of age. The patient was well 6 months postoperatively, without unilateral pulmonary hypertension.     

Asthma-like Attacks Resulting from the Isolated Congenital Left Pulmonary Artery Agenesis with Right Main Bronchus Stenosis

A 5-year-old girl with isolated congenital left pulmonary artery agenesis suffered from recurrent attacks of dyspnea and right-sided pneumonia due to the stenosis of the right main bronchus. The division of ligamentum of the ductus arteriosus and suspension of the right pulmonary artery resulted in the disappearance of symptoms. It is notable that the compression of contralateral bronchus by the remaining pulmonary artery can cause respiratory symptoms in patients with isolated unilateral pulmonary artery agenesis.     

Single Origin of Right and Left Pulmonary Artery Branches from Ascending Aorta with Nonbranching Main Pulmonary Artery: Relevance to a New Understanding of Truncus Arteriosus

We report the third known case of origin of the right and left pulmonary artery branches from the ascending aorta via a short common pulmonary artery. A large unbranching main pulmonary artery opened through a patent ductus arteriosus into the descending thoracic aorta. Preductal coarctation of the aorta and multiple congenital anomalies were also present. This rare cardiovascular malformation facilitates a new anatomic and developmental understanding of truncus arteriosus.     

Absent pulmonary artery

Summary Cardiac catheterization was performed in nine patients with unilateral absence of a pulmonary artery. Aortography revealed a diverticulum of the innominate artery in five of six patients in whom the aortic arch and absent pulmonary artery were on opposite sides. It is suggested that the diverticulum indicates fetal systemic blood supply to the affected lung through the distal part of the sixth aortic arch, which—consisting of ductal tissue—obliterated after birth, leaving the diverticulum where the ductus joins the innominate artery.     

Congenital Pulmonary Steal Phenomenon Associated with Tetralogy of Fallot, Right Aortic Arch, and Isolation of the Left Subclavian Artery

Isolation of the left subclavian artery has been reported in cases of tetralogy of Fallot with a right aortic arch. As the isolated left subclavian artery is supplied by the left vertebral artery (in which the blood flow direction is inverted), this anomaly is usually responsible for a congenital subclavian steal phenomenon. In our case the isolated left subclavian artery was connected by a patent ductus arteriosus to the main pulmonary artery. Therefore the left vertebral artery did not supply the distal left subclavian artery but supplied the main pulmonary artery (congenital pulmonary artery steal).     

Atypical Bland–White–Garland Syndrome with Stenosis of the Origin of the Left Coronary Artery: Catheter Intervention After Mammary Artery Bypass Stenosis and Residual Fistula to the Pulmonary Trunk

A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland–White–Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.     

Angiographic evidence of absent ductus arteriosus in severe right ventricular outflow obstruction

Summary The angiocardiograms of 5 newborn infants with autopsy and/or surgically-proven congenital absence of the ductus arteriosus (ADA) and right ventricular outflow obstruction (Group A), and of 14 neonates with pulmonary atresia complex and patent ductus arteriosus (Group B) were reviewed. Aortic size was similar in both groups; however, the diameters of the right and left pulmonary arteries were much smaller in Group A than in Group B (right pulmonary artery: 2.6 vs 4.5 mm,P<0.005; left pulmonary artery: 2.5 vs 4.3 mm,P<0.005). Extensive bronchial collaterals were observed in Group A but not in Group B. Tricuspid aortic valve stenosis was present in 2 patients in Group A but in none in Group B. The diagnosis of ADA may be made in newborn infants with severe right ventricular outflow obstruction if the angiocardiograms reveal hypoplasia of the pulmonary arteries, extensive bronchial collaterals, and nonvisualization of the ductus arteriosus. Other suggestive features include aortic valve stenosis and/or right aortic arch with aberrant left subclavian artery.Supported in part by Grant RR-305 from the General Clinical Research Centers Program of the Division of Research Resources and by Grant HL-07605 from the National Heart, Lung and Blood Institute. National Institutes of Health, Bethesda, Maryland     

Closure of the ductus arteriosus: determinant factor in the appearance of transient peripheral pulmonary stenosis of the neonate

We speculated that a relationship may exist between transient peripheral pulmonary stenosis and the closure of the ductus arteriosus. Fifty preterm infants had pulmonary artery and ductal color Doppler flow velocity assessments performed before and after closure of the ductus arteriosus. No flow turbulence or increase in velocity was observed immediately after birth, although a significant discrepancy in size was observed between the main pulmonary artery and its two branches. After closure of the ductus, 15 infants had signs of transient peripheral pulmonary stenosis of the left pulmonary artery in association with a significant decrease of diameter at the origin of the same artery. In all 50 infants, no significant gradient was observed in the right pulmonary artery. We conclude that, at least in the preterm infant, transient peripheral pulmonary stenosis is not present at birth but is an acquired phenomenon closely related to closure of the ductus arteriosus.     

Left-to-right shunt lesions

A left-to-right shunt lesions exists when blood from the left atrium, left ventricle, or aorta transits to the right atrium or its tributaries, the right ventricle, or the pulmonary artery. This article discusses: the incidence, types, embryology, clinical presentations, physical examinations, electrocardiographic features, chest radiographs, echocardiographic and cardiac catheterization issues, treatment, natural history of atrial septal defects, and outcomes of treatment of atrial septal defects, ventricular septal defects, patent ductus arteriosus, and endocardial cushion defects.     

Subclavian Steal Syndrome in Anomalous Connection of the Left Subclavian Artery to the Pulmonary Artery in D-Transposition of the Great Arteries

Several reports have documented the occurrence of an isolated left subclavian artery in association with both tetralogy of Fallot and double-outlet right ventricle. In certain cases a congenital subclavian or pulmonary artery steal syndrome exists in which the left subclavian artery is connected to the main pulmonary artery via a ductus arteriosus. We describe a subclavian steal syndrome secondary to anomalous origin of the left subclavian artery from the pulmonary artery in d-transposition of the great arteries in a patient with Spondylocostal dysostosis (SCD). Cardiac anomalies are rare in SCD and this constellation of findings have not previously been described.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Associated with Ventricular Septal Defect and Mitral Stenosis

We report a case of a child with ventricular septal defect, mitral stenosis, and patent ductus arteriosus, who was also found to have anomalous origin of the left coronary artery from the pulmonary artery. Preoperative diagnosis allowed successful surgical correction.     

Critical Ebstein Anomaly in a Fetus Successfully Managed by Elective Preterm Delivery and Surgical Intervention Without Delay After Birth

This report describes a case of Ebstein anomaly in a fetus with cardiomegaly, severe tricuspid regurgitation, pulmonary regurgitation, and retrograde ductal flow that showed a marked increase in the size of the right atrium with advancing gestational age. Elective preterm delivery was performed at 35 weeks gestation. The prostaglandin E1 infusion resulted in more pronounced systemic hypotension and acidosis secondary to circular shunt across the patent ductus arteriosus as well as pulmonary regurgitation and tricuspid regurgitation. Emergency surgical intervention consisting of main pulmonary artery ligation, ductus arteriosus ligation, central shunt creation, and plication of the right atrium without cardiopulmonary bypass was performed 4 h after birth. At the age of 16 days, the Starnes procedure was performed. The infant’s postoperative course was uneventful. A fetus that has Ebstein anomaly associated with pulmonary regurgitation is at risk for circular shunt across the patent ductus arteriosus after delivery. Planned delivery and surgical intervention without delay after birth are useful for the treatment of such cases.     

Aortography by countercurrent injection via the radial artery in infants with congenital heart disease

Summary Aortography by countercurrent injection into the radial artery was performed for analysis of aortic arch anomalies in 12 infants with congenital heart disease. Eleven aortograms were obtained without complications, but one failed. Coarctation of the aorta, patent ductus arteriosus, aberrant origin of the right subclavian artery, interrupted aortic arch, anomalous origin of the right pulmonary artery from the ascending aorta, and hypoplastic ascending aorta were demonstrated by this method. Patency of the reconstructed aortic arch was confirmed in the postoperative study of the case of interrupted aortic arch. Aortography by countercurrent injection via the radial artery affords an easy, safe, and simple bedside means for analysis of aortic arch anomalies without retrograde arterial catheterization. The latter is occasionally complicated by thrombosis of the artery in small infants.