Brojendra Agarwala

Spontaneous Acquisition of Discontinuous Pulmonary Arteries

BACKGROUND Discontinuous pulmonary arteries have been considered a rare complication of systemic-to-pulmonary shunt operations. We report a series of children who spontaneously acquired pulmonary artery discontinuity. METHODS All children from 1989 through 1995 with congenital pulmonary atresia were reviewed. RESULTS Pulmonary artery discontinuity developed in 29% (15 patients), none related to shunt operation. In 6 of 15 patients, the neonatal angiogram showed a pattern that seemed to predict subsequent discontinuity; in 9 of 15, pulmonary arteriography was normal at birth. Two clinical patterns were identified: an early rapid acquisition of discontinuity within hours to days, and a delayed, more subtle development that occurred over months. Eight of 15 have died. Pathologic studies in 6 children showed ductal tissue extending along and into the pulmonary artery wall as well as intimal hypertrophic reaction and maladaptive remodeling. CONCLUSIONS Children with congenital pulmonary atresia may experience spontaneous acquisition of pulmonary artery discontinuity. Ductal tissue is responsible for local pulmonary artery distortion and discontinuity; this may be exacerbated by previous prostaglandin E1 administration. Clinical algorithms are suggested for patients with pulmonary atresia.

Complete heart block from mycoplasma pneumoniae infection

SummaryComplete heart block (CHB) in infants and children is usually congenital. Nonsurgical acquired CHB is rare. Occasionally, transient acquired CHB is seen in association with viral myocarditis. We describe here an unusual case of transient CHB in a 12-year-old boy with endomyocardial biopsy-proven myocarditis and evidence ofMycoplasma pneumoniae infection.

Acute myocardial infarction in a teenager due to Adderall XR.

Adderall XR is commonly prescribed for children and adolescents with attention deficit/hyperactivity disorder. We present a case of a 15-year-old male who suffered a myocardial infarction after starting Adderall XR. Patient was otherwise in good health with no previous cardiac abnormalities. Cardiac catheterization was normal, and etiology was presumed to be secondary to acute vasospasm. The patient improved with cessation of medication. Physicians need to carefully screen patients for cardiac abnormalities prior to starting amphetamine-based medications.

Surgical issues in giant right ventricular fibroma

Cardiac fibroma is a rare and benign tumor usually seen in infants and children. We report a 3-year-old asymptomatic child who presented with a giant right ventricular (RV) fibroma. He underwent complete surgical resection and is doing well at follow-up. Because of the size of the tumor and the potential need for resection of RV free wall, it is essential to have contingency plans to deal with postoperative RV failure.

Intracardiac Lymphoma in a Child: Successful Treatment with Chemotherapy Alone

Abstract. A 5-year-old female child with history of non-Hodgkins lymphoma presented with cough and palpitation. On physical examination a cardiac tumor plop was heard. Paroxysmal supraventricular tachycardia was noted on the electrocardiogram. Transthoracic echocardiogram revealed multiple large tumor masses within the right and the left atrium. The right atrial tumor was flopping back and forth at the tricuspid valve orifice. The tumor resolved completely with chemotherapy without any surgical intervention.

Acquired pulmonary stenosis: Ultrasonographic diagnosis

A 15-year-old boy presented with chest pain and a new heart murmur. The clinical diagnosis of pulmonary stenosis was confirmed by two-dimensional and Doppler echocardiographic examinations. A large, encapsulated, partly solid and partly cystic tumor in the anterior mediastinum, visualized by ultrasonography, was compressing the main pulmonary artery and producing the right ventricular outflow tract obstruction. The tumor was removed surgically and was found to be a benign teratoma. Postoperative ultrasound examination of the right ventricular outflow tract showed no evidence of obstruction. We conclude that: (1) there are subtle clinical diagnostic differences between intrinsic and extrinsic pulmonary stenosis; (2) ultrasound examination can make cardiac catheterization unnecessary; and (3) relief of the extrinsic cause of pulmonary stenosis should be curative.

Aortic origin of the RPA: Immediate resolution of severe pulmonary artery hypertension by surgical repair

SummaryAortic origin of the right pulmonary artery (AORPA) is a rare condition requiring a high index of suspicion for diagnosis as the usual signs of structural heart disease are often absent. Ultrasound examination can strongly suggest the condition but can easily be misinterpreted. Infants should be considered “operable” even when the resistance calculations predict otherwise as there appears to be a large component of immediately reversible pulmonary artery hypertension. With technical skills learned from arterial switch operations (for transposition of the great arteries), surgical repositioning of the RPA should be possible in nearly all cases without the use of a tube graft.

Congestive Heart Failure Caused by Wilms’ Tumor

A 2-year-old child with unilateral Wilms’ tumor presented with congestive heart failure (CHF). The heart failure was secondary to severe hypertension from hyperreninemia. After surgical removal of the tumor the CHF disappeared. This case is the first one reported of a child presenting with CHF due to unilateral Wilms’ tumor.

Intrapericardial Teratoma in a Newborn

Intrapericardial teratomas are very rare and most are benign in nature. Neonates can remain asymptomatic; however, they can present with respiratory distress from compression and pericardial tamponade. In a fetus, nonimmune hydrops fetalis could be a finding. The case presented here is of a neonate with intrapericardial teratoma who underwent surgical resection with excellent result. A 2-week-old baby boy underwent median thoracotomy resection of a capsulated intrapericardial multicystic tumor. This was adherent to the pericardium superiorly and to the adventitia of the aorta and main pulmonary artery posteriorly (Figs. 1, 2). This was confirmed as a teratoma by histopathology. On the prenatal echocardiogram, an anterior mediastinal cystic mass was seen. The infant was delivered following a full-term twin pregnancy by caesarian section with a birth weight of 2.3 kg and Apgar score of 7 and 9 at 1 and 5 min, respectively. At birth, the infant remained asymptomatic with normal vital signs and normal physical examination. Prior to surgery, a two-dimensional (2D) echocardiogram (Fig. 3) showed a multicystic capsulated tumor within the pericardium superior to the ascending aorta and to the left of the superior vena cava. A computed tomography (CT) scan of the mediastinum (Fig. 4) revealed an intrapericardial capsulated cystic tumor surrounded by thymic tissue. Eight percent of all mediastenal tumors are benign teratomas [3]. Intrapericardial teratomas accounts for 0.003% [2]. The patient can be asymptomatic or can present with cardiorespiratory distress and pericardial temponade. Fetal presentation can be that of nonimmune hydrops fetalis [5]. Teratoma originates form totipotent cells from Hansen’s node or from primitive germ cells during their migration from the yolk sac [6]. It consists of complex multiple tissues. The presence of all three layers of cells (i.e., endoderm, mesoderm, and ectoderm) is not necessary to make a diagnosis [4]. Gonzalez-Crussi [1] has graded teratomas as follows: 0 = mature (benign), 1 = immature (probably

Kawasaki disease: giant aneurysm with a large thrombus of the left coronary artery

We report a six-month-old febrile infant presenting with stridor. Later on, he developed typical Kawasaki disease with giant aneurysm of the coronary artery with thrombosis that resolved with an aggressive anticoagulation therapy. The giant aneurysm still persisted a year later. Respiratory illness with stridor is an unusual presentation of Kawasaki disease.