Early echocardiographic and pulmonary function findings in idiopathic scoliosis.

Thirty-six children and adolescents with early stages of idiopathic scoliosis underwent evaluation by echocardiography and pulmonary function testing. Mildly increased pulmonary vascular resistance was inferred from an elevated ratio of right preejection period to right ventricular ejection time, an increased right ventricular dimension, and a decreased left ventricular dimension. Since neither decreased arterial oxygen saturation nor increased end-tidal expired carbon dioxide partial pressure was seen, desaturation and hypoventilation should not account for these abnormalities. Pulmonary function parameters showed no distinct patterns of abnormality. Even though the patients were divided into two groups by severity of spinal curvature, the cardiopulmonary measures did not correlate with thoracic deformity. Billowing of the mitral leaflets, termed mitral valve prolapse, was demonstrated in 25% of the subjects. Our findings suggest that cardiopulmonary and thoracic changes in idiopathic scoliosis may develop in parallel and may be expressions of a common collagen defect. However, study of sleep and exercise arterial saturation may be required to rule out intermittent hypoxemia as a precipitating factor of cor pulmonale in scoliosis.

Aortic valve replacement in childhood: evaluation of left ventricular function by electrocardiography, echocardiography and graded exercise testing.

Preoperative and postoperative left ventricular (LV) performance was evaluated noninvasively in 15 children who survived aortic valve replacement (AVR). The noninvasive evaluation included electrocardiography, M-mode echocardiography, and graded exercise testing. Clinically, there was dramatic improvement postoperatively: All but 2 patients were asymptomatic. No conduction defects or arrhythmias were detected preoperatively; however, in the late postoperative period there was a variety of intraventricular conduction abnormalities, myocardial infarctions, and ventricular or supraventricular arrhythmias. On M-mode echocardiography, children with aortic stenosis continued to have increased LV mass postoperatively. Shortening fraction and left-sided systolic time intervals returned to normal. The children with aortic regurgitation also had persistently abnormal LV mass on echocardiography postoperatively. Exercise data indicated no improvement in working capacity after AVR. Also, 9 children (63%) continued to have ST-segment depression with maximal exercise. These data indicate that AVR does not result in a return to normal of myocardial performance in children with severe aortic valve disease.

The univentricular heart: combined 2-dimensional-pulsed Doppler (duplex) echocardiographic evaluation.

Two-dimensional echocardiographic-pulsed Doppler (2D-PD) evaluation was performed in 14 patients with a univentricular heart. Accurate information on ventricular structure, atrioventricular valve status, and great vessel anatomy and function was obtained. The characteristic morphologic features were verified in each case by angiography or autopsy, or both. In several patients, 2D-PD evaluation provided more accurate anatomic and functional information than did cardiac angiography in standard views, particularly with regard to atrioventricular valve function. Four children with pulmonary atresia and 4 with pulmonary stenosis were identified prospectively. No patient had 2D-PD evidence of aortic obstruction. Four children had typical 2D-PD patterns consistent with atrioventricular valve regurgitation. Thus, 2D echocardiography coupled with a range-gated Doppler system will improve recognition of patients with a univentricular heart and permit thorough evaluation of specific anatomic details.

Severe pulmonary hypertension associated with a small ventricular septal defect.

A case of progressive pulmonary hypertension in a child with a small ventricular septal defect is presented. Natural history studies have indicated that children with small ventricular septal defects can be followed conservatively. This case represents a contradiction to that rule and suggests that further study must be directed toward defining the etiology of pulmonary hypertension in patients with congenital heart disease.

Echocardiographic and hemodynamic findings in isolated symptomatic coarctation of the aorta in infancy.

Echocardiographic findings and cardiac catheterization data were evaluated in 18 infants less than 1 year old in order to define anatomical or pathophysiological features that were associated with early cardiac decompensation. The infants could be divided into three groups: Group I (10 patients) had left ventricular dilatation and depressed contractility in response to the severe systemic hypertension. Group II (3 patients) had marked myocardial hypertrophy In response to the systemic hypertension. Group III (5 patients) were the youngest patients and had findings of right ventricular volume overload and pulmonary hypertension. This study demonstrates that, in early infancy, the ventricular response to simple coarctation of the aorta is variable in infants in a state of cardiac decompensation. The different echocardiographic and hemodynamic findings may be a consequence of the lesion exerting its influence at various stages of the patients' intrauterine or postnatal life. In most patients, resection of the coarctation results in rapid normalization of the echocardiographic findings.

Incidence of mitral valve prolapse in adolescent scoliosis and thoracic hypokyphosis.

Seventy-four patients with adolescent scoliosis underwent cardiac examination and M-mode echocardiography to detect the presence of mitral valve prolapse (MVP). Twenty-one (28%) had echocardiographic evidence of MVP, whereas 18 had auscultatory findings of a nonejection click or late systolic murmur. A subset of 41 patients had a family history of scoliosis and 37% had MVP. The incidence of MVP increased to 41% when a first degree relative, such as a sibling, parent, or offspring, had scoliosis. Thirty-six patients with scoliosis had additional thoracic hypokyphosis (straight back) and 13 (36%) had MVP. The incidence of MVP was 48% when the scoliosis and hypokyphosis were hereditary and increased to 53% when a familial history of skeletal abnormality was present. This study indicates a high incidence of MVP in patients with scoliosis and hypokyphosis, especially when the cardiac and skeletal systems may be affected by a generalized soft-tissue defect.

Concealed left atrial membrane: pitfalls in the diagnosis of cor triatriatum and supravalve mitral ring.

Cor triatriatum and supravalve mitral ring are forms of congenital left ventricular inflow obstruction produced by membranes within the left atrium. Typically, these defects occur as isolated anomalies with manifestations of pulmonary venous obstruction. Four children are presented whose left atrial membrane was associated with other significant cardiac defects, including, in one patient each, simple coarctation of the aorta, sinus venosus atrial septal defect, tricuspid atresia and complex coarctation of the aorta syndrome. The patient with the latter defect had undergone previous pulmonary arterial banding. None of these patients demonstrated significant pulmonary venous obstruction at cardiac catheterization. All patients had a normal value for either pulmonary arterial diastolic or pulmonary arterial wedge pressure. Three mechanisms explained the lack of pulmonary venous obstruction: (1) a large cross-sectional area of membrane openings, (2) an atrial septal defect that was confined to the pulmonary venous chamber and decompressed it by allowing blood to escape into the right atrium, and (3) decreased pulmonary blood flow. The diagnosis was facilitated by two dimensional echocardiography. Accurate diagnosis of left atrial membrane in the setting of other cardiac defects is of practical significance because pulmonary venous obstruction may occur after surgery for the associated defects.

Ventricular interdependence in severe cystic fibrosis. A two-dimensional echocardiographic study.

A variety of mechanisms have been implicated in the development of left ventricular dysfunction in patients with chronic cor pulmonale. A two-dimensional echocardiographic study of cystic fibrosis (CF) patients with severe cor pulmonale was undertaken to evaluate the effects of long-term pulmonary abnormalities on right and left ventricular geometry. Ten patients with severe obstructive pulmonary disease secondary to CF underwent evaluation by a mechanical sector scanner from the long axis, short axis, and four chambered views. All patients manifested right heart failure. Eight had clinical scores less than 40 and died within six months of the initial examination. All patients were receiving diuretics, and six were taking digoxin at the time of the study. The most striking echographic feature was flattening or compression of the left ventricle along its minor dimension by a massively dilated right ventricle. Compression of the left ventricle and additional abnormalities of interventricular septal motion resulted in dyskinetic contraction and relaxation that could contribute to a diminished stroke volume. Massive right ventricular enlargement appears to be a major factor producing left ventricular dysfunction in chronic cor pulmonale.

Heart failure in cystic fibrosis. Treatment and prognosis of cor pulmonale with failure of the right side of the heart.

Failure of the right side of the heart with cardiac dilation and fluid retention occurred in 55 of 170 patients who died of cystic fibrosis; six patients survive. All had severe hypoxia, but 24% had normal PaCO2. Cardiac catheterization showed high mean pulmonary artery pressure and resistance. Pulmonary artery wedge pressure was greater than 12 mm Hg in 40% of the patients. Mean survival was eight months. Male survival was significantly better than female survival. Digitalis treatment was of no clear benefit. Tolazoline hydrochloride was also ineffective. Recent medical advances have not substantially affected prognosis.

Echocardiographic abnormalities in patients with cystic fibrosis.

An echographic study was undertaken to evaluate left (LV) and right ventricular (RV) function in 30 patients with cystic fibrosis. Echographic recording of the pulmonary and aortic valve echogram permitted measurement of the phases of right and left ventricular systole. The ratio of the LV preejection period/LV ejection time (LPEP/LVET) and shortening of the LV internal dimension %SID was employed to reflect LV function, while RV preejection period/RV ejection time (RPEP/RVET) has excellent correlation with pulmonary artery diastolic pressure. RPEP/RVET and two other echographic measurements, right ventricular wall (RVW) and internal dimension (RVD) were compared with pulmonary function tests and clinical scores. RPEP/RVET correlated well with percent vital capacity(%VC), r = -0.73, percent residual volume (%RVol) r = +0.72, and clinical score, r = -0.77. Multilinear regression of RPEP/RVET, RVD, and RVW improved correlation for %VC (r = -0.80), %RVol, r = +0.82, and clinical score, r = -0.84. Patients in overt right heart failure exhibited elevated RPEP/RVET (mean = 0.48) when compared to patients not in right heart failure (mean = .33). Marked diminution of LV function was present in two patients. A variety of cardiovascular abnormalities were demonstrated echographically and were valuable in assessing the degree of cardiac involvement in patients with cystic fibrosis.