Libman–Sacks endocarditis may be the first manifestation of systemic lupus erythematosus. The risk of its occurrence increases with the co-existence of the anti-phospholipid syndrome. Changes usually involve the mitral valve and the aortic valve. In this report, we present a case of Libman–Sacks endocarditis of the tricuspid valve in a teenage girl.

Total anomalous systemic venous drainage is an extremely rare congenital heart defect. In this study we describe an 11-year-old girl who presented with a history of fatigue and central cyanosis that she had had since early childhood with unremarkable precordial examination results. Investigations revealed left heterotaxy with all systemic venous drainage to the left-sided atrium with non-compaction of the left ventricle.

We report the case of a young woman with continuing haemoptysis, pulmonary atresia, previous shunt surgery, and pulmonary hypertension. She was not suitable for further surgery or for therapeutic embolisation of bronchial vessels. Treatment with tranexamic acid resolved the haemoptysis.

In a hemodynamically compromised neonate with complete transposition and intact ventricular septum (concordant atrioventricular and discordant ventriculo-arterial connections), it became necessary to control severe congestive heart failure. The arterial switch operation had been delayed because of intracranial bleeding and subsequent coagulopathy. The cardiac failure was reversed by surgical banding of the patent arterial duct. The arterial switch was performed successfully one week later.

A young man had anomalous connection of the veins draining the upper lobe of the left lung to both a left-sided vertical vein and the left atrium. The ratio of pulmonary to systemic flows was 1.7 : 1, and he was symptomatic with evidence of volume overload of the right heart. He was successfully treated by percutaneous placement of an Amplatzer ductal occlusion device into the vertical vein.

Cardiac catheterisation continues to play an important role in the long-term management of patients with common arterial trunk and transposition of the great arteries. Although non-invasive imaging has largely eliminated the need for diagnostic catheterisation in newborns with these congenital cardiac lesions, cardiac catheterisation is an important tool for the diagnosis of a variety of problems encountered after surgical intervention, and allows interventions to be performed when feasible. We review the indications for cardiac catheterisation and describe the specifics for various interventional procedures for these patients in this manuscript.

Transcatheter pulmonary valve replacement is fast becoming an accepted alternative to repeat surgical pulmonary valve replacement for selected patients and therefore a complementary strategy in the long-term management of those requiring surgical pulmonary valve replacement. With a combined surgical and percutaneous approach, late morbidity for some of these patients may be diminished. This manuscript will review the current indications for this procedure, its limitations, and its benefits.

This study reviews the results of the surgical management of 154 cases of ruptured aneurysm of the sinus of Valsalva. Of the patients0 73% were male, with an average age of 28 years. An associated ventricular septal defect was found in 40% and 23% had aortic valvar regurgitation. The aneurysms originated from the right coronary sinus in 79% and from the non-coronary sinus in the remainders. The aneurysms ruptured into the right ventricle in 73%, into the right atrium in 27% and into the left ventricle in less than 1%. Operative mortality was 4.5%. Long-term follow-up was achieved in 80% of patients, with a mean duration of 5.7 years and a range from two months to 29 years. Preoperative aortic regurgitation and preoperative functional class (NYHA III or IV) were both predictive of a worse long-term outcome. The optimal surgical approach was closure of the distal end of the fistula by direct suture together with reinforcement of the aortic sinus with a Dacron patch.

We describe the finding of unsuspected aortic coarctation, discovered by catheterisation at 4 months, in a neonate first daignosed on the fourth day of life as having tetralogy of Fallot with pulmonary atresia and major aorto-to-pulmonary collateral arteries. This previously unreported combination contravenes the established theory of patterns of flow during fetal life, and presented difficult issues in clinical management.

Tetralogy of Fallot is often found in association with a wide variety of other cardiac lesions, but is rarely found in association with lesions causing obstruction to the left ventricular inflow or outflow. Subaortic stenosis has only rarely been reported in association with tetralogy of Fallot. We report a patient with Marfan syndrome who underwent repair of tetralogy of Fallot at five years of age. Discrete and progressive subaortic stenosis developed two years after the surgical correction, in a previously normal and unobstructed left ventricular outflow tract. Surgical removal of the acquired fibrous subaortic shelf was successful. Clinical signs of obstruction within the left ventricular outflow tract after surgical repair of tetralogy of Fallot should prompt further investigation to exclude the onset of acquired subaortic stenosis.

Transcatheter pulmonary valve insertion is the most important advance in congenital interventional cardiology since atrial septal defect devices became commonly available 15 years ago. It has changed the way we look at a number of diverse diagnoses and changes how we plan, diagnose, operate, and follow-up patients. It has changed how we counsel families expecting a child that may benefit from it. Expanded use of the Melody® valve, outside its United States Food and Drug Administration approved indications, has helped numerous additional patients. The use of transcatheter pulmonary valve insertion in selected patients following surgical Gore-tex® bileaflet in valve right ventricular outflow tract reconstruction and those with a history of prior small homograft conduits will be discussed.

This manuscript presents a technically straightforward technique to allow for mitral valve repair in the patient with rheumatic mitral stenosis. This non-resection technique allows for the correction of both mitral stenosis and regurgitation without requiring complex subvalvar procedures and eliminates the concerns for postoperative systolic anterior motion. The authors feel this three-part technique of bi-commissural release, anterior leaflet augmentation, and oversized annuloplasty may allow for a more reproducible approach to repair of the rheumatic mitral valve.

We report a case of 2:1 atrioventricular block associated with acquired long QT syndrome. A newborn presented to our neonatal intensive care unit with intermittent bradycardia due to 2:1 atrioventricular block. Initial evaluation showed QT prolongation and significant electrolytic abnormalities. After correction of the electrolytic imbalance, the QT interval normalized and atrioventricular block resolved. Compared to congenital long QT syndrome with 2:1 atrioventricular block, acquired long QT syndrome with comparable atrioventricular block has a benign prognosis, provided treatment is initiated quickly.

A symptomatic neonate was referred for aortic coarctation and, at cineangiography, was shown to have an interruption of the aortic arch at the isthmus together with coarctation related to a persistent fifth aortic arch. Balloon dilation of the coarctation was performed, but the coarctation was still evident after six months. A graft was then interposed between the left subclavian artery and the descending aorta. Balloon dilation was repeated five years later leaving a residual gradient of 11 mm Hg.

We report a case of endocarditis of a transcatheter pulmonary valve-in-valve in a 14-year-old boy with tetralogy of Fallot. He presented with recurrent low-grade fevers, lethargy, and anorexia. Multiple blood cultures grew a gram-positive rod, Corynebacterium pseudodiphtheriticum. He was taken to the operating room for removal of the vegetative endocarditis and pulmonary valve replacement.