Steroid-induced hypertrophic cardiomyopathy following stem cell transplantation in a neonate: a case report.

We report a case of severe left ventricular outflow tract obstruction complicating steroid therapy in an infant undergoing allogeneic transplant in the first few weeks of life for treatment of Krabbe's disease. While this complication is well known to those treating premature infants, it has not been reported in the stem cell transplant setting. For young infants undergoing allogeneic transplant who require steroid therapy, cardiac monitoring after 2--3 weeks of therapy is recommended.

Arcade mitral valve and anomalous left coronary artery originating from the pulmonary artery.

The use of color Doppler interrogation significantly improves accuracy in the evaluation of coronary artery anatomy. This was demonstrated in a patient in whom the left coronary artery originated from the posterior and medial aspect of the pulmonary artery, a particularly difficult coronary anomaly to detect by 2-dimensional imaging. In addition to the coronary artery anomaly, this patient also had an arcade mitral valve.

Clinical spectrum of restrictive cardiomyopathy in children.

We reviewed the clinical spectrum and possible prognostic factors in 14 children with restrictive cardiomyopathy. The patients were not homogeneous in clinical presentation or morphology. The mortality rate was high: 21.4% at 1 year and 50% at 2 years after presentation. Younger patients with respiratory symptoms, thromboembolism, increased cardiothoracic ratio on chest radiogram or patients with endocardial fibroelastosis appear to have a worse prognosis and orthotopic cardiac transplantation may be indicated.

Five-loop coil occlusion of patent ductus arteriosus prevents recurrence of shunt at follow-up.

Recent reports suggest reopening of the patent ductus arteriosus (PDA) after complete occlusion with three-loop Gianturco coils. We hypothesize that five-loop coils may produce a larger thrombus than three-loop coils, which will result in no or less probability of recanalization of PDA during follow-up. This study is designed to test this hypothesis. Follow-up echocardiographic and Doppler data of 30 patients who underwent five-loop coil occlusion of small to medium-sized PDA during a 33-month period ending December 1998 were examined. Thirty patients had no residual shunt on echo Doppler study on the day following the procedure and were followed for 6 to 30 months (median, 12) after coil implantation. At the last follow-up study, none of the patients had a residual shunt and left atrial size decreased. Careful pulsed, continuous wave, and color Doppler interrogation of left/main pulmonary artery junction and proximal descending aorta did not reveal any evidence for obstruction. The follow-up data suggest that complete occlusion of small- to medium-sized PDAs is feasible with five-loop coils without evidence for recanalization at a mean follow-up of 12 months. Much longer (2 to 5 years) follow-up data may be necessary to confirm these observations. We speculate that a greater degree of thrombosis is produced within the ductus by the five-loop coils, which in turn may be responsible for lack of shunt recurrence. We recommend use of five-loop instead of three-loop coils for transcatheter occlusion of small- to medium-sized PDAs.

Main coronary artery and coronary ostial stenosis in children: detection by transthoracic color flow and pulsed Doppler echocardiography.

Coronary artery stenosis (CAS) and coronary ostial stenosis (COS) are potentially life-threatening conditions. The echocardiographic diagnosis of CAS and COS in children has not been described. We report on the transthoracic echocardiography (TTE) findings of CAS and COS in children. Six patients, aged 1 week to 12 years, with clinically confirmed COS (n = 5) and CAS (n = l) were diagnosed by TTE. Their echocardiographic findings were compared with 26 healthy control subjects of a similar age range. Left COS was associated with an aberrant left coronary artery (CA) from the contralateral aortic sinus (n = 2), an intramural left CA with d-transposition of the great vessels (n = l), and supravalvular aortic stenosis (n = l). Right COS was present in a patient with aortic valvular stenosis. Acquired left main CAS was diagnosed in the sixth patient 3 years after orthotopic heart transplantation. Coronary ostial stenosis was recognized when a color flow acceleration signal was present proximal to and extending into the coronary ostium (CO). Coronary artery stenosis was detected when a coarctated color flow stream was present within the stenosed CA segment with turbulent distal flow. These findings were not detected in the control cohort who demonstrated laminar CA and CO flow signals. All patients had increased spectral velocity in the CA distal to the stenosed segment (patients = 50 +/- 5 cm/s, controls = 24 +/- 6 cm/sec; P <.01). Delayed peak diastolic velocity seemed to indicate severe stenosis. We conclude that (1) CO acceleration signals and turbulent coarctated CA flow signals are abnormal findings in TTE coronary Doppler assessment. They indicate COS and CAS, respectively. (2) Knowledge of the normal TTE CA flow velocity patterns is essential so that abnormal velocity signals such as seen with CAS and COS can be recognized and a timely diagnosis made.

Coronary artery to pulmonary artery collaterals after heart transplantation.

Collaterals are described between coronary and pulmonary arteries after orthotopic heart transplantation. It is likely the collaterals developed as a result of adhesions in the pericardial space. This is a previously unrecognized complication of heart transplantation with potential clinical implications, as coronary to extracardiac artery collaterals have been shown to cause myocardial ischemia.

Cyclosphosphamide/prednisone for combination immunosuppression and therapy of lymphoproliferative disease.

Post-transplant lymphoproliferative disease (PTLD) is a well-known complication of solid organ transplantation. While this disorder can often be controlled by decreasing immunosuppression, it is frequently difficult to control the resultant rejection. This case exemplifies this dilemma. To solve this problem, cyclosphosphamide (600 mg/m2) and prednisone (2 mg/kg times 5 days) were given every 3 weeks to simultaneously treat PTLD and provide immunosuppression.

Balloon angioplasty of long-segment aortic coarctation in the neonate.

It is generally believed that balloon angioplasty of diffuse, long-segment aortic coarctation is not effective. In this report, we describe two neonates with diffuse, long-segment coarctation in association with complex congenital heart defects in whom we were successful in effectively treating coarctation with transumbilical artery balloon angioplasty. Based on this experience, it may be concluded that balloon angioplasty of long-segment coarctation in neonates is feasible and effective, but confirmation in a larger group of patients may be necessary prior to general adoption of this concept.

Transthoracic Doppler echocardiography of normally originating coronary arteries in children.

Transthoracic Doppler color flow and spectral velocity patterns of normal coronary arteries in children have not been well studied. We designed this study to evaluate coronary artery flow velocity characteristics in normal and hypertrophied hearts. Sixty-eight children with optimal two-dimensional echocardiographic images of the left coronary artery (LCA) and right coronary artery (RCA) were prospectively studied. The heart was normal in 45 children, and 23 had left and/or right ventricular hypertrophy assessed by echocardiography (mean age 5.8 versus 5.2 years, p = NS). Color flow signals were detected in the LCA in 63(92%) of the 68 children studied, and pulsed Doppler spectral waveforms were recorded in 47 (69%). The latter were recorded in 26 (58%) of 45 normal children and in 21 (91%) of 23 children with left ventricular hypertrophy. Diastolic RCA flow signals were detected mostly in those with right ventricular hypertrophy (10 of 10). Higher levels of LCA maximum diastolic velocity (42 +/- 23 versus 24 +/- 6 cm/sec, p = 0.0004), increased diastolic flow (16 +/- 15 versus 6 +/- 4 ml/min, p = 0.01), and delayed time to peak diastolic velocity expressed as a percentage of diastolic spectral duration (38% +/- 14% versus 20% +/- 8%, p = 0.0001) were observed in children with left ventricular hypertrophy than in those in normal children. A strong correlation was present between Doppler-derived LCA flow and left ventricular mass/m2 (r = 0.7, p = 0.001). In normal hearts, LCA spectral velocity pattern did not change with increasing age, but the time velocity integral became progressively larger, resulting in a strong correlation with weight (p < 0.001, r = 0.78). This study demonstrates (1) LCA flow signals can be detected and quantitated in the majority of children with and those without left ventricular hypertrophy. (2) Left ventricular hypertrophy is associated with increased LCA flow, higher diastolic velocity, and delayed peak diastolic velocity. (3) RCA flow signals are mostly detected when there is right ventricular hypertrophy. Studies on larger groups of patients are needed to further confirm our observations and to enhance understanding of coronary artery flow reserve.

Familial syndrome of progressive arterial occlusive disease consistent with fibromuscular dysplasia, hypertension, congenital cardiac defects, bone fragility, brachysyndactyly, and learning disabilities.

We report on 4 of 9 sibs with a syndrome of stenosis of the renal arteries and chronic hypertension, variable stenosis or occlusion of cerebral, abdominal and probably coronary arteries due to suspected fibromuscular dysplasia, congenital cardiac abnormalities, brachydactyly and syndactyly of the hands and feet, and increased bone fragility consistent with a mild form of osteogenesis imperfecta. Three affected individuals have had mild to moderate learning disabilities. The parents and the remaining 5 sibs have normal hands and feet and no history of excessive fractures. Individual components of this syndrome may appear as isolated conditions, including fibromuscular dysplasia, brachydactyly, syndactyly, and osteogenesis imperfecta, and are autosomal dominant traits in many cases. Explanations for this familial occurrence include autosomal recessive inheritance, autosomal dominant inheritance with decreased penetrance, or parental gonadal mosaicism for a mutation involving a single gene or several contiguous genes.

Chest pain in children.

Chronic chest pain is a complaint that frequently prompts referral to pediatric cardiology clinics although very few pediatric patients with this symptom will be found to have cardiac disease. This review will discuss the common non-cardiac and cardiac causes of chest pain. Guidelines on the office management of this group of patients will be presented as well as the indications for referring patients for evaluation and treatment by the subspecialist.

Effectiveness of five-loop coils to occlude patent ductus arteriosus.

Coil occlusion of patent ductus arteriosus with 5-loop coils was undertaken in 10 patients without coil embolizations, and with 90% immediate occlusion and 100% occlusion at follow-up. We conclude that 5-loop coil occlusion of patent ductus arteriosus is safe and effective.

Transcatheter management of neonates with pulmonary atresia and intact ventricular septum.

This report describes a 1 day-old infant with valvar pulmonary atresia with intact ventricular septum in whom we were successful in performing transcatheter guidewire perforation and balloon pulmonary valvuloplasty to establish right ventricle-to-pulmonary artery continuity and flow. Also described is implantation of a 4 mm coronary stent into ductus arteriosus in lieu of surgical aortopulmonary shunt to treat pulmonary oligemia and systemic arterial hypoxemia. Details of transcatheter guidewire perforation are presented and it is suggested that this method be used as an alternative to Laser/radio frequency wires, especially in the absence of approval of the latter wires by the regulatory agencies. Stenting of the ductus may be considered an alternative to surgical aortopulmonary shunt. Role of transcatheter technology in the management of selected patients with pulmonary atresia and intact ventricular septum is discussed.

Transient and persistent expansions of large granular lymphocytes (LGL) and NK-associated (NKa) cells: the Yorkshire Leukaemia Group Study.

A survey of 870 different adult blood samples (primarily from patients with non-haematological disorders) found that 269 (31%) had increased proportions (> 25%) and/or absolute numbers (> 1.0 x 10(9)/l) of morphologically-defined large granular lymphocytes (LGL), and/or phenotypically-defined NK-associated (NKa) cells. Of these, 112 were re-analysed at least 6 months after initial presentation and were classified as 'persistent' (92/112) or 'transient' (20/112) according to whether or not the original abnormality was still present. Lymphocyte counts in most patients with persistent abnormalities were within normal limits (18/92) or slightly increased (68/92), with only six having a lymphocytosis exceeding 10.0 x 10(9)/l. With the exception of five persistent LGL expansions in which the granular lymphocytes did not express NKa determinants (designated LGL+NKa-), the remaining 87 cases could be phenotypically grouped according to their primary abnormality as CD8+NKa+ (n = 33), CD4+ NKa+ (n = 14), CD8dim+NKa+ (n = 7) or CD8-NKa+ (n = 33). TCR genotypic studies in 58 patients showed that the 16 patients with rearranged TCR components were restricted to the CD8+NKa+ group and that, in most of these, the CD8+ fraction showed abnormal relative CD16/CD56 expression. Persistent neutropenia (n = 15) also appeared to be associated with primary abnormalities of CD8+NKa+ cells (12/15), with 10 of these additionally showing rearranged TCR genes. In contrast, persistently increased CD8dim+NKa+ and CD8-NKa+ components did not appear to phenotypically differ from their corresponding 'counterparts' in normal bloods or in patients with transient LGL/NKa+ abnormalities. This survey has therefore established that persistent LGL/NKa+ abnormalities are considerably more common than suggested in published work, that a high proportion of patients with expanded CD8+NKa+ components, with quite diverse clinical histories, show evidence of clonal lymphoid populations, and that the clonal nature of such disorders appears to be associated with abnormal NKa phenotypic patterns.

Pediatric cardiac rehabilitation.

Sixteen patients participated in a Pediatric/Young Adult Cardiac Rehabilitation Program that included exercise training, education about cardiovascular diseases, dietary counseling, and counseling on stress management. Seven patients completed the program, and complete data were available on six. The subjects demonstrated significant changes in their hemodynamics and exercise tolerance after completing the program. Resting blood pressure decreased by 7%, from 119 +/- 12 to 111 +/- 10 mm Hg; peak oxygen consumption increased by 20%, from 31.9 +/- 4.3 mL/kg of body weight per minute to 38.4 +/- 6.0 mL/kg of body weight per minute; and exercise treadmill time increased by 21%, from 8.5 +/- 1.4 to 10.3 +/- 1.0 minutes. No complications occurred during exercise training or testing. Supervised exercise training at moderate intensity is safe and produces significant and beneficial changes in hemodynamics and exercise time in children with cardiac disease.