Pulmonary artery pseudoaneurysms are uncommon. They may occur secondary to trauma, infectious diseases, vasculitis syndromes, neoplasms, congenital diseases, and pulmonary hypertension. Due to increasing number of cardiac interventions, iatrogenic complications are among the major causes of pulmonary artery pseudoaneurysms.

In this report, we present a 6-month-old patient with pulmonary pseudoaneurysm that occurred following pulmonary balloon angioplasty for the relief of a pulmonary band.

Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

Images of the diagnosis and correction of a pseudo-aneurysm at the right ventricular outflow tract, one of the rarest complications of Tetralogy of Fallot surgical correction.

Abnormal small fistulous flows in the pulmonary artery were detected on routine transthoracic echocardiography in asymptomatic patients by colour Doppler echocardiography. The most likely diagnosis is small coronary artery-pulmonary artery fistulas. We evaluated the clinical, echocardiographic, and follow-up findings of 101 patients. The mean age at first echocardiographic evaluation was 4.3 ± 4.2 years. In 79 (78.2%) of the patients, fistula flow in the pulmonary artery was diagnosed at the first presentation and the remaining 22 patients (21.8%) were diagnosed between the 2nd and 10th examination. The echocardiography indication was cardiac murmur in 42 (41.6%), routine cardiac control in 30 (29.7%), additional CHD in 14 (13.8%), non-specific chest pain in 11 (10.9%), suspicion of inflammatory heart disease in 2 (2%), and syncope in 2 (2%) patients. In 70 (69.3%) patients, fistulous flow was located in the anterior aspect of the main pulmonary artery, in 23 (22.8%) patients on the aortic side of the pulmonary artery and in 8 (7.9%) patients on the right pulmonary artery. Additional cardiac anomalies were ventricular septal defect in 8, patent ductus arteriosus in 6, atrial septal defect in 5, mitral valve prolapse in 4, coarctation of aorta in 4, bicuspid aortic valve in 3, and Kawasaki syndrome in 1 patient. Sixty-four patients (63.3%) were followed during a mean of 52.6 ± 43.7 months. Spontaneous closure was detected in only three patients; the others remained almost unchanged during the follow-up. Since the fistulas are thin and hemodynamically insignificant, echocardiography is an appropriate method to monitor these patients without performing any invasive diagnostic procedures for the fistula source.

Disconnection of a pulmonary artery needs early surgical treatment in order to support the growth of the vessel. However, owing to the high rate of re-stenosis after traditional surgical reconstruction, we developed a hybrid approach involving the creation of pulmonary artery continuity by using autologous or heterologous tissue supported by stent implantation.

In this study, we describe a very rare case of anomalous left coronary artery arising from the right pulmonary artery in association with coarctation of the aorta. A 3-month-old boy presented with refractory congestive heart failure since 20 days after birth. The initial echocardiography suggested the diagnosis of left coronary artery-to-right pulmonary artery fistula associated with coarctation; however, selective coronary angiography demonstrated the rare anomaly of the left coronary artery arising from the right pulmonary artery. Subsequently, he underwent successful transcatheter balloon angioplasty for aortic coarctation and surgical repair of left coronary artery re-implantation.

A 20-day-old girl was referred to our clinic for systolic murmur, cyanosis, and dyspnoea with feeding. Echocardiography revealed an atretic aortic valve. CT angiography scan revealed that the left common carotid artery originated from the distal main pulmonary artery. The plan was patent ductus artriosus stenting and bilateral pulmonary artery banding and then follow-up for any possible future intervention.

Isolated left subclavian artery from the pulmonary trunk is a rare congenital cardiovascular malformation. In this report, we present the images of ascending aortic aneurysm and left subclavian artery originating from the pulmonary artery in a 4-year-old girl in addition to her congenital cardiac pathology.

This study describes single-unit experience and explores risk stratification, with protocolised inter-stage cardiac magnetic resonance imaging for infants with hypoplastic left heart syndrome. Survival was retrospectively analysed among the cohort of locally followed survivors of Norwood Stage I procedure, who underwent magnetic resonance imaging under general anaesthesia from 2003 to 2008. This included 32 patients: 17 with Sano conduit and 15 with arterio-pulmonary shunt. The median (inter-quartile range) age and weight at scan were 3.1 (2.6–4.6) months and 5.0 (4.5–5.3) kilograms, respectively. Using morphologic definitions, the median coarctation index was 0.71 (0.57–0.83). The degree of proximal right and left pulmonary artery narrowing was 25% (14–44%) and 25% (11–50%), respectively. The median right ventricular ejection fraction was 54% (48–59%). The ejection fraction was not related to the coarctation index or to pulmonary artery narrowing. Patients were followed up for a median of 19.2 (10.8–46.0) months, during which 13 (41%) had an intervention in addition to routine Norwood Stage II surgery and seven died. Risk of death was related to reduced right ventricular ejection fraction, with a hazard ratio of 0.91 (95% confidence interval 0.85–0.98, p = 0.02), and the cumulative number of focal stenoses of neo-aortic arch and pulmonary arteries (hazard ratio 2.71, 95% confidence interval 1.14–6.44, p = 0.02). Conclusions: In addition to comprehensive three-dimensional morphologic imaging, inter-stage cardiac magnetic resonance imaging provides a ventricular functional index that may predict outcome in patients with hypoplastic left heart syndrome. Measures to preserve right ventricular systolic function and relieve stenoses are paramount within the complex management strategies for these patients.

So as to understand better the pathogenesis of enlargement or hypoplasia of pulmonary arteries in congenital heart disease, we studied the dimensions of the pulmonary arteries in 74 fetuses with congenital heart disease induced by administration of bis-diamine to pregnant rats. The congenital malformations induced included 12 with large ventricular septal defect, 17 with tetralogy of Fallot, 15 with tetralogy together with severe valvar pulmonary stenosis and absence of the arterial duct, 17 with tetralogy with absent pulmonary valve syndrome and absence of the arterial duct, and 13 with common arterial trunk with a confluent segment supplying the pulmonary arteries. For comparison, 16 fetuses of the same gestational age with normal hearts were studied. After rapid whole-body freezing on the 21st day of gestation, the fetuses were studied by means of serial cross-sectional photographs of the thorax. The diameter of the right pulmonary artery of the fetus was of comparable dimensions in the normal hearts (480±10 µm) (mean±SEM), those with ventricular septal defects (470±10 µm), common arterial trunk (520±20 µm), and tetralogy of Fallot (500±10 µm). These findings suggest that the commonly observed enlargement of the right pulmonary artery in patients with ventricular septal defect and common arterial trunk, and hypoplasia of the right pulmonary artery in tetralogy of Fallot, occur postnatally in response to abnormal postnatal pulmonary blood flow.

We describe an infant with congenitally corrected transposition, ventricular septal defect and severe pulmonary stenosis. The heart occupied a midline position. Extension of ductal tissue had resulted in occlusion of the left pulmonary artery. As far as we are aware, this is the first report of an association of coarctation of the left pulmonary artery with corrected transposition.

Mycotic aneurysms of the pulmonary trunk are a rare but recognized complication of infective endocarditis. They are usually associated with infected congenital heart lesions, and especially with persistent patency of the arterial duct. This report deals with a patient with a persistent duct and aortic coarctation who developed a mycotic aneurysm of the pulmonary trunk secondary to infective endocarditis. The lesion was evaluated before and after surgery by magnetic resonance imaging.