Aaron R. Levin

Left ventricular abnormality with late mitral insufficiency and abnormal electrocardiogram.

Abstract An abnormal contraction that alters the contour of the left ventricle only during systole but not in diastole and results in mild mitral insufficiency has been demonstrated in 6 girls with apical systolic click, late systolic murmur and T wave inversion. They have remained asymptomatic during a period of follow-up studies ranging from 3 to 17 years. Their young age and the absence of myocarditis, pericarditis and coronary arterial abnormality suggest that they have a congenital anomaly in a localized area of the left ventricular myocardium which results in late systolic dysfunction of the mitral apparatus. We believe that the findings in this group constitute a distinctive syndrome among the heterogeneous causes of mitral insufficiency late in systole.

Eccentric Ventricular Hypertrophy in Familial and Sporadic Instances of 46 XX, XY Turner Phenotype

This study of individuals with familial and sporadically occurring 46 XX or XY Turner phenotype documented a wide range of right- and left-sided cardiovascular abnormalities and a previously unreported eccentric hypertrophy of the left ventricle.A mother and five of her seven children had abnormal cardiovascular findings. Five had an abnormal electrocardiogram with frontal ÂQRS of −60° to ±180° and rS or rsr′ in V1 and rS, qrS, or qRS in V5. Catheterization demonstrated the following anomalies: coarctation of the aorta in three, valvular aortic stenosis in one, pulmonary valvular insufficiency with atrial septal defect in one, and pulmonary arterial branch stenosis in one child. All six had a similar abnormality of the left ventricle on angiocardiography. During systole and also in diastole the cavity was encroached on in its superolateral and posteroinferior aspects. Septal hypertrophy altered right ventricular contour in two.A similarly abnormal electrocardiogram and left ventricle were found in four unrelated individuals with the XX, XY Turner phenotype. Three had pulmonary stenosis; in two there was an associated septal defect. The fourth, with no associated cardiac defect, died in heart failure at 5 months of age. At necropsy she had marked eccentric biventricular hypertrophy, chiefly involving the left ventricle so that the chamber was reduced to a slitlike cavity. The hypertrophied septum bulged into the right ventricular outflow tract.A number of cardiovascular anomalies occur in familial and sporadic instances of this syndrome; eccentric ventricular hypertrophy recognizable by an electrocardiographic abnormality seems to be a distinctive cardiac lesion in the XX, XY Turner phenotype. Except for the unusual and, we believe, characteristic ECG, there was no clinical clue on physical examination or cardiac series of chest roentgenograms to suggest the presence of eccentric left ventricular hypertrophy. We recommend that selective left ventricular angiocardiography be performed when patients with the Turner phenotype undergo diagnostic cardiac catheterization, especially when the frontal ÂQRS is superiorly directed.

Assessment of Left Ventricular Function in Secundum Atrial Septal Defect: Evaluation by Determination of Volume, Pressure, and External Systolic Time Indices

Extract: Left ventricular function and volume data from 17 control subjects and 27 young patients with secundum atrial septal defect (ASD) without overt left or right ventricular failure were compared. ASD patients were subdivided in low shunt (Qp/Qs < 2.0) and high shunt (Qp/Qs ≥ 2.0) groups. Mean left ventricular (LV) stroke volume was significantly less in ASD patients (46 ± 16 ml/m2 in the low shunt and 44 ± 9 ml/m2 in high shunt group) compared with control patients (51 ± 13 ml/m2, P < 0.01 and P < 0.02, respectively). There was no significant difference in mean left ventricular end-diastolic volume (LVEDV) between any group of patients (control subjects 67 ± 17 ml/m2; low shunt ASD 66 ± 17 ml/m2, and high shunt ASD 62 ± 12 ml/m2). High shunt ASD had a significantly lower cardiac index compared with control patients (5.0 liters/min/m2 vs. 5.9 liters/min/m2, P < 0.02). Both low shunt and high shunt ASD showed significantly lower stroke work indices than control subjects (42 ± 13 GmM/m2 and 37 ± 8 GmM/m2 compared with 51 ± 14 GmM/m2, P < 0.05 and P <0.001, respectively) but only the high shunt group had a significantly lower peak systolic pressure (94 ± 12 mm Hg vs. 109 ± 11 mm Hg for control patients, P < 0.01). There was no significant difference between the control and ASD groups in LV end-diastolic, mean right atrial, right ventricular end-diastolic, and pulmonary pressures.External systolic time intervals were compared in 5 control and 12 ASD patients. There was no significant difference between the two groups of patients in absolute values or indices for pre-ejection period, ejection time, or electromechanical systole. However, the ratio of the pre-ejection period index to left ventricular ejection time index (PEPI/LVETI) was significantly higher in ASD patients (P < 0.05).In young subjects with large shunt ASD, certain indicators of left ventricular function are depressed. Evaluation of PEPI/LVETI may allow noninvasive determination of LV function.Speculation: Previous studies have shown that left ventricular failure may occur in adults with right ventricular volume overload. The finding of left ventricular dysfunction in children with right ventricular volume overload because of atrial septal defect would confirm the relationship between right ventricular volume overload and secondary functional changes occurring in the left ventricle. Such changes in left ventricular function may be primary or due to alterations in left ventricular geometry because of increased right ventricular enlargement.

Congenital aortic Regurgitation: Natural history and management

OBJECTIVES AND BACKGROUND Congenital aortic regurgitation is rare as an isolated lesion. We describe seven children with no physical features of the Marfan syndrome in the patients or their families and no other cardiac lesions who had congenital valvular aortic regurgitation. METHODS From 1954 to the present, seven children with auscultatory and physiologic characteristics of aortic regurgitation were evaluated for a total of 108 patient-years. We report on their natural history, clinical and laboratory findings, management and outcome. RESULTS In five of the seven children congenital aortic regurgitation was diagnosed in infancy. In four, progressive severity of the regurgitation led to valve replacement at age 3, 10, 15 and 20 years, respectively, and to resection of an aneurysm of the ascending aorta in the 10-year old patient. Two patients had cystic medial necrosis on aortic biopsy. One of these patients died after reoperation for dissecting aneurysm of the thoracic aorta at 22 years of age; the other died after dissection and rupture of the ascending aorta at age 25 years. After obstructing pannus developed, the 3-year old patient underwent replacement of the St. Jude valve at age 10 years. The other three patients were asymptomatic at last follow-up at age 8, 10 and 20 years, respectively. CONCLUSIONS Supportive management is recommended until it becomes necessary to intervene surgically when regurgitation becomes severe. The need for surgical treatment is indicated by the appearance of a diastolic thrill, left ventricular strain on the electrocardiogram or other evidence of left ventricular dysfunction on the echocardiogram or exercise stress testing by treadmill or radionuclide cineangiocardiography. Close follow-up of these patients is important to detect progression of aortic regurgitation, especially in the presence of cystic medial necrosis.

Physiologic studies on infants with Wilson-Mikity syndrome: Ventilation-perfusion abnormalities and cardiac catheterization angiography†

Abnormalities of ventilation and perfusion were found in infants with the Wilson-Mikity syndrome. Intrapulmonary shunting and maldistribution of ventilation and perfusion were noted. Three patients were subjected to cardiac catheterization and pulmonary hypertension was detected. The increased pulmonary vascular resistance was unresponsive to oxygen, suggesting permanent damage to the pulmonary capillary bed. Angiocardiography showed changes typical of pulmonary hypertension.

Wide splitting of the second heart sound without demonstrable heart disease.

Abstract Twelve subjects had wide splitting of the second heart sound (S 2 ) during quiet respiration, during a variety of respiratory maneuvers, and in the supine and sitting position. Cardiac hemodynamics were normal. Neither the mechanism nor the incidence of such splitting in the absence of significant heart disease is clear.

Transient complete heart block following percutaneous balloon pulmonary valvuloplasty: Treatment with systemic corticosteroids

SummaryBalloon pulmonary valvuloplasty is a safe and effective treatment for congenital pulmonic valve stenosis. This report describes a child who developed complete atrioventricular (AV) block following balloon pulmonary valvuloplasty. The child was treated with a 10-day course of systemic corticosteroids. The heart block gradually resolved during the first week following the procedure and has not recurred. Heart block is a known complication of right heart catheterization and has been described following balloon pulmonary valvuloplasty. Systemic corticosteroids have been used to treat AV block of various etiologies and may have contributed to the childs recovery in this case.

The Appearance of the Left Ventricle in Noonan's Syndrome

Twenty patients with Noonans syndrome underwent heart catheterization and angiocardiography. The incidence of congenital cardiac lesions in these patients is reviewed. The predominant lesion was an eccentric hypertrophy affecting the superior portion of the anterior wall, the septum and∕or the diaphragmatic portion of the left ventricle, In all but one instance, this hypertrophy did not produce a hemodynamic burden, but its presence must be recognized because it can explain an electrocardiographic pattern usually associated with other specific congenital cardiac lesions.