Chest
Volume 93, Issue 1, January 1988, Pages 119-122
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Cor Pulmonale Due to Adenoidal or Tonsillar Hypertrophy or Both in Children: Noninvasive Diagnosis and Follow-Up

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Four children, aged 1 to 3½, were first seen with cor pulmonale, pulmonary edema and severe respiratory distress due to chronic upper airway obstruction secondary to adenoidal or tonsillar hypertrophy or both. Arterial blood gas values, electrocardiograms and chest x-ray films were compatible with cor pulmonale. Echocardiography (four cases) and radionuclide angiography (two cases) showed severe right ventricular and right atrial dilatation with reduced right ventricular ejection fraction. Following surgery, all four children improved; their echocardiographic and radionuclide findings returned to normal. Cardiac catheterization, traditionally peformed in such cases, was unnecessary. The diagnosis and follow-up of this syndrome are adequately performed noninvasively.

Section snippets

Patients

Four children, aged 1 to 3½ years, were first seen with acute respiratory distress. In all four, parents reported symptoms compatible with upper airway obstruction almost from birth, including mouth breathing, noisy breathing while sleeping, frequent upper respiratory infections, chronic rhinorrhea, poor appetites and episodes of sleep apnea with cyanosis. The main clinical and laboratory findings on admission are summarized in Table 1. Three boys and one girl were first seen with severe

Techniques

M-mode and two-dimensional echocardiography was performed on all four patients using an Aloka Echocardiograph SSD-110S and a Sony Betamax video cassette recorder. Radionuclide angiography was performed on patients 1 and 4 using the method reported by Parrish et al.6 These noninvasive procedures were performed shortly after admission and prior to intubation, during supplemental oxygen administration. Because of their severe distress, we were unable to transfer patients 2 and 3 to radionuclide

Results

Echocardiography showed severe dilatation of the right atrium and of the right ventricle (RV) in all four patients (Table 2).7 This severe dilatation of the RV produced marked displacement of the interventricular septum (IVS) posteriorly and to the left, causing a marked alteration in normal left ventricle (LV) geometry. Instead of the normal round configuration of the LV, we observed a markedly flattened appearance in systole which was best observed in the parasternal short axis view (Fig 1,

Discussion

Pulmonary hypertension, cor pulmonale and even pulmonary edema have been reported in children with chronic upper airway obstruction.1, 2, 3, 4, 5 Most patients were first seen with features now referred to as the “obstructive sleep apnea syndrome,” with signs of respiratory and heart failure. Cardiac catheterization was frequently performed in these children to exclude congenital heart disease or cardiomyopathy or both.1, 2, 3, 4 The clinical features of upper airway obstruction and their

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Manuscript received January 30; revision accepted May 4.

Reprint requests: Dr. Sofer, Soroka Medical Center, PO Box 151, Beer Sheva, Israel 84-101

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