Indian Pediatrics 1999;36:307-310 

Adenosine Infusion in the Management of a Micropremi Neonate with Pulmonary Hypertension

Alka Kalgutkar
Shobha Pandit
Vijayshri M. Pethe
J.A. Mondkar
Armida Fernandes

From the Departments of Pathology and Pediatrics*, L.T.M. Medical College and General Hospital, Sion, Mumbai 400 022, India.

Reprint requests: Dr. Alka Kalgutkar, 02/12, 'Kinara', 358 M.C.G.B. Colony, A.G. Khan Road, Worli, Mumbai 400 018, India.

Manuscript Received: September 6, 1997; Initial review completed: October 2, 1997;
Revision Accepted: July 22, 1998

 

Neoplasms of the heart are rare at all age groups. Although the frequency of primary cardiac neoplasms in the pediatric age group is very low, rhabdomyomas account for about 45% of all cardiac tumors, occurring most commonly in the neonatal period(1).

Case Report

A term male newborn presented on the 5th day of the life with breathlessness and refusal of feeds for two days. The newborn was tachypneic and had respiratory distress, but was not cyanotic. Arterial gasometry showed pH of 7.26,pC0₂-40, p0₂-36, HC0₃-14.6, O₂ Saturation-82% and base excess 9. X-ray chest showed bilateral prominent bronchovascular markings. A clinical diagnosis of septicemia with pneumonia was made. The patient was managed with intravenous fluids, omnatax and phenobarbitone. The patient died within 12 hours of admission. Echocardiography could not be done, but an autopsy was performed.

Necropsy revealed multiple, well-circumscribed, non-encapsulated translucent nodules of varying sizes in the left atrium and left ventricle. The largest nodule measured 3.0 x 2.5 cm in size, obstructing the orifice of the mitral valve, in turn causing dilation of the left atrium. Another intramural tumor was seen in the lateral wall of the left ventricle. The right atrium and right ventricle were unremarkable. The brain was edematous. There was a tiny, discrete sub-ependymal nodule, about 1 cm in diameter in the wall of lateral ventricle. The lungs were boggy and edematous. The liver showed nutmeg appearance. The spleen was grossly unremarkable. Histopathology of the cardiac tumor revealed vacuolated cells, with no features of anaplasia. Features of tuberous, sclerosis were noted in sections of nodular area in the brain. An exceptionally rare finding of splenic histiocytosis was also noted.

Discussion

Cardiac rhabdomyomas have interested the medical profession due to their rarity. One hundred cases have been described in the literature till 1976 with addition of ten more cases recently. Fenoglio, et al, published a comprehensive review of 36 cases highlighting the clinico-pathologic correlation and electron microscopic features(2). Fetal manifestations include polyhydramnios, intra-uterine death and non-immune fetal hydrops. Cardiomegaly, systolic murmur of changing intensity and cyanosis are the major signs of cardiac failure in children. Congestive. cardiac failure is noted only in patients with intracavitatory rhabdomyomas, suggesting that it is due to hemodynamic obstruction, rather than replacement of the myocardium by the tumor(3). Multiple rhabdomyomas with exclusively histologic manifestation have been recently described(4). They have also been reported as incidental and un-suspected autopsy findings(2,5,6). Two-dimensional and Doppler Echocardiography are very useful techniques for diagnosis, including the smallest intramural lesion that is likely to escape detection other-wise and in the longitudinal follow-up of these pateints(6). Compared to echocardiography, MRI demonstrates a clear plane of demarcation between the mass and cardiac wall, making surgical resection a viable life saving option(7).

A unique association of cardiac rhabdomyomas with splenic histiocytosis, as seen in our case, has been reported by several authors(5). An enzymatic abnormality allowing accumulation of glycogen has been suggested for its pathogenesis.

Rhabdomyomas are associated with tuberous sclerosis in more than 50% of cases. This was first described by Boumeville in 1880(8). In fact, fetal cardiac rhabdomyoma has been used as a marker for prenatal detection of tuberous sclerosis. Prenatal MRI is useful in diagnosing tuberous sclerosis in fetuses with cardiac rhabdomyomas(9).

Cardiac rhabdomyomas are benign, tend to regress over time and never metastatize. A neonate with intracavitatory rhabdomyomas showing hemodynamic compromise is an ideal candidate for surgical resection of the tumor. Current techniques of profound hypothermia and cardiopulmonary bypass allow an access to these tumors(6). Orthotopic cardiac trans- plantation is indicated in infants of rhabdomyoma presenting with severe myocardial ischemia(10). Neonatal pulmonary autograft implantation has been described for replacing the aortic root, when it is involved by the tumor. This may - be done with scarcity of neonatal cardiac donors(11). Termination of pregnancy may be an option when tuberous sclerosis is detected during prenatal check-ups.

The association of tuberous sclerosis with cardiac rhabdomyomas raises important questions regarding the quality of life for neonates surviving tumor resection. Although tuberous sclerosis is transmitted through a dominant gene, the expressivity and penetrance are variable. Genetic counselling is difficult but important because of the poor prognosis of the disease.

 References