Elfriede Pahl

Extracorporeal membrane oxygenation for postcardiotomy cardiogenic shock in children

Size limitations and technical barriers prohibit the use of many conventional mechanical circulatory support systems for postcardiotomy ventricular dysfunction in pediatric populations. Extracorporeal membrane oxygenation (ECMO), frequently used to treat neonatal respiratory failure, can provide cardiac support and is effective treatment of postoperative myocardial failure in children. From 1981 to 1987, 10 patients aged 2 days to 5 years were maintained on ECMO for 15 to 144 hours (mean duration, 92 +/- 16 hours) after cardiotomy. Operative procedures included repair of tetralogy of Fallot (2 patients), closure of a ventricular septal defect (2), the Senning procedure for transposition of the great arteries (1 patient), repair of interrupted aortic arch with closure of a ventricular septal defect (1), repair of a partial atrioventricular septal defect (2), closure of a ventricular septal defect with excision of an anomalous muscle bundle (1), and the Fontan procedure (1). Venoarterial ECMO was established in all 10 children. Six patients underwent transthoracic right atrium-ascending aorta cannulation, 3 had right internal jugular vein-right common carotid artery cannulation through a cervical incision, and 1 had right internal jugular vein-left axillary artery cannulation. Eight of the 10 patients were successfully weaned from ECMO, and 7 are long-term survivors. There were 3 deaths; 1 was caused by cardiac and acute renal failure complicated by sepsis two days after decannulation, another occurred 19 days after atrioventricular septal defect repair, and 1 was caused by massive pulmonary hemorrhage. Major hemorrhage developed in 3 patients while on ECMO; 2 required premature decannulation for mediastinal bleeding from operative sites and ultimately survived, and 1 died of respiratory failure as a result of endobronchial bleeding. We conclude that the use of ECMO in pediatric populations for transient postoperative ventricular dysfunction improves survival with limited overall morbidity.

Coronary arteriosclerosis in pediatric heart transplant survivors: Limitation of long-term survival

Because coronary atherosclerosis after heart transplantation has been a limiting problem in long-term survival of adults, we reviewed the coronary angiograms, and autopsy data when available, from 21 of 30 children who underwent orthotopic heart transplantation and survived the perioperative period. Six patients had coronary atherosclerosis, and five of these patients died 6 months to 3 years after heart transplantation. The late deaths were sudden and unexpected. Coronary angiography demonstrated several types of lesions, including concentric narrowing, tubular segmental lesions, and abrupt obliteration of major coronary vessels. Risk factors assessed included hypertension, hyperlipidemia, cytomegalovirus infection, type of immunosuppressive regimen, number of rejection episodes, and major histocompatibility antigen mismatches. Only the frequency and duration of rejection episodes seemed to be more prevalent in the patients in whom coronary atherosclerosis developed. Despite the benefits of heart transplantation in treating children with end-stage heart disease, coronary atherosclerosis may limit long-term survival. We suggest that these children should undergo serial coronary angiography to identify those at risk for subsequent events related to coronary artery disease.

The value of angiography in the follow-up of coronary involvement in mucocutaneous lymph node syndrome (Kawasaki disease)☆

From September 1976 to August 1988, 205 patients with mucocutaneous lymph node syndrome (Kawasaki disease) were evaluated; 29 were suspected of having coronary artery abnormalities because of either abnormalities on echocardiography (n = 21), congestive heart failure (n = 3), prolonged fever (n = 3) or cardiac arrest (n = 2). All 29 underwent cardiac catheterization and 22 were found to have coronary artery abnormalities. An additional 4 patients were diagnosed solely by autopsy; therefore, 26 patients (12%) in this series had coronary artery abnormalities. The clearance of contrast medium from the coronary arteries after aortography was significantly prolonged in patients with coronary artery aneurysms as compared with that in a control group (p less than 0.001). All patients exhibited delayed clearance of contrast medium on the initial aortogram as compared with that on follow-up studies (p less than 0.01). Of the 22 patients with angiographically documented coronary artery abnormalities, 19 had right and 20 had left coronary artery involvement; the majority had diffuse involvement of both vessels. In 15 patients who underwent follow-up catheterization 6 months to 7 years after initial study, complete resolution was observed in only 37% of affected coronary artery segments. In four patients, three of whom were asymptomatic, severely stenotic or occluded coronary artery segments were found on late catheterization. Coronary artery bypass surgery was performed in two of these patients. Echocardiography did not detect any of these stenoses. Although echocardiography is important for initial diagnosis and serial evaluation, angiography is essential to fully define the nature and extent of coronary artery lesions, particularly if stenosis is present.

Fistulous communications between a solitary coronary artery and the pulmonary arteries as the primary source of pulmonary blood supply in tetralogy of fallot with pulmonary valve atresia

Abstract A communication between the coronary and pulmonary arteries, described previously only in isolated cases1–3 and a series of 5 patients in which the communication was called an “aortopulmonary tunnel,”4 is a rare source of pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia. Between 1954 and 1987, we encountered 4 patients with tetralogy of Fallot and pulmonary atresia in whom the pulmonary flow to one or both lungs was via a fistulous communication between a coronary artery and the intrapericardial pulmonary arteries. They form the basis of this report.

Diagnostic echocardiographic features of the sinus venosus defect.

To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Childrens Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.

Spontaneous closure of the ventricular component of an atrioventricular septal defect.

Abstract It is well recognized that perimembranous ventricular septal defects close or diminish in size spontaneously due to the development of fibrous tissue tags, which are usually termed “aneurysms of the membranous septum.” Morphologic examination, however, has shown conclusively that they are almost always derived from the leaflets of the tricuspid valve. 1 Equally well described, although certainly less well recognized, are the “aneurysmal-like” structures that may be derived from the bridging leaflets in the setting of an atrioventricular (AV) septal defect. The angiomorphologic correlation study of Kudo et al 2 established the frequent existence of such pouches in the setting of so-called “ostium primum” defects, but suggested that they did not exist with common AV valve orifice. We now report a patient with AV septal defect and common orifice in whom ventricular shunting was virtually abolished concomitant with the development of pouches from the bridging leaflets that blocked the septal defect. This report illustrates the angiographic findings in this patient together with the morphologic features of a heart specimen exhibiting a similar malformation.

Usefulness of the internal jugular venous route for cardiac catheterization in children

Abstract Cardiac catheterization is an important tool in the diagnosis and management of heart disease in children. The femoral artery and vein are the usual routes of access for this procedure. In the past, use of the internal jugular vein for cardiac catheterization has been reserved for those children in whom femoral venous access was limited by obstruction, such as ileofemoral venous thrombosis or congenital absence of the hepatic portion of the inferior vena cava. Latson et al 1 reported use of this vein in 14 pediatric patients, in each for reasons of either inferior vena caval obstruction or the desire to maintain a central venous line after catheterization. At the Childrens Hospital of Pittsburgh, the internal jugular vein has been used as a primary site for cardiac catheterization in patients undergoing endomyocardial biopsy after cardiac transplantation. This report describes our experience and technique.

Controversies in the medical management of Kawasaki disease

Summary Mucocutaneous lymph node syndrome, now called Kawasaki disease, affects children of all ages and races. The cause of this systemic vasculitis is unknown, thus the diagnosis is based solely on clinical criteria. Coronary arteritis and the formation of coronary arterial aneurysms are the most serious early cardiac complications, while long term sequels, such as coronary stenosis and myocardial infarction, may occur. Current medical therapy is aimed solely at preventing or decreasing the occurrence of these cardiovascular complications in patients with this disease. Past treatment included antibiotics, steroids, and nonsteroidal anti-inflammatory agents. Aspirin remains the most widely used drug, although the use of intravenous gammaglobulin has also become common. When used in combination, these two agents may reduce the incidence of coronary arterial aneurysms. The optimal dosage and duration of treatment has not yet been determined.

Experience with a newly developed pericardiocentesis set

Abstract Pericardiocentesis is a widely used therapeutic and diagnostic procedure. Although a limited number of pericardiocentesis sets are commercially available, none has been developed for use in children. This report describes experience with a newly developed pericardiocentesis set, specifically designed for pediatric patients.

Postoperative cardiopulmonary failure in tetralogy of Fallot with absent pulmonary valve syndrome treated by extracorporeal membrane oxygenation

When tetralogy of Fallot with absent pulmonary valve syndrome leads to respiratory failure in early infancy, total repair has been advocated. Respiratory insufficiency may persist despite a surgically adequate repair. An infant with this syndrome underwent total surgical correction. Extracorporeal membrane oxygenation was employed in the early postoperative period for intractable pulmonary insufficiency and myocardial dysfunction.