Multiple Cardiac Anomalies Research Articles

Transaortic Resection of the Subaortic Membrane: Treatment for Subvalvular Aortic Stenosis

In an attempt to better understand congenital subaortic stenosis, we reviewed 19 consecutive pediatric patients undergoing surgery for this problem between 1973 and 1984. Preoperative cardiac catheterization demonstrated subvalvular stenosis in all patients and associated anomalies in 11 patients. All patients underwent transaortic resection of the subaortic membrane. Five patients underwent concurrent additional cardiac procedures. An 8-month-old infant with multiple cardiac anomalies was the only operative mortality. Follow-up extended to 16 years, with a mean of 6.9 years. In five symptomatic patients, cardiac catheterization revealed a mean systolic pressure gradient of 33.0 +/- 31.94 mm Hg 24.7 months (mean) after surgery. Two of these patients (11.1 percent of survivors) required reoperation. Twelve of the remaining 13 asymptomatic patients underwent echocardiographic follow-up, and 4 were found to have recurrent stenosis with 1 requiring surgery (5.6 percent of survivors). Our experience shows that transaortic resection of the subaortic membrane is an acceptable treatment for subvalvular aortic stenosis, but is associated with a high incidence of recurrence requiring reoperation (3 of 18 or 16.7 percent). Consequently, it is reasonable to consider the role of alternative therapies which may help prevent recurrence in selected cases.

Visceral anomalies in the Apert syndrome

We report on visceral anomalies found in 136 patients with Apert syndrome. Autopsies were only performed on 12 of these cases. Thus, the percentage of anomalies found in our patients should be considered a minimum estimate because of the possibility of clinically silent visceral anomalies, minor internal anomalies, and anatomic variations. Cardiovascular and genitourinary anomalies were found most commonly, occurring in 10% and 9.6%, respectively. As expected, complex and multiple cardiac anomalies were frequently associated with early death. Among genitourinary anomalies, hydronephrosis (3%) and cryptorchidism (4.5%, n = 66 males) occurred most commonly. In contrast, anomalies of the respiratory system (1.5%) and gastrointestinal anomalies (1.5%) occurred with lower frequency. The finding of a solid cartilaginous trachea is particularly important because no case was diagnosed during life but rather, only at autopsy. Because cardiovascular and genitourinary anomalies occur with significant frequency, they should be considered in the workup of all Apert newborn infants. We also recommend MRI study of the trachea in any infant with signs and symptoms of lower respiratory compromise.

Multiple births and cardiac anomalies in the bottle-nosed dolphin.

A bottle-nosed dolphin, Tursiops truncatus, delivered a stillborn male fetus, and three months later, died in dystocia. The second fetus, a female, had a transposed pulmonary artery and aorta and an interventricular septal defect.

Mosaic Turner's Syndrome With Unusual Manifestations

<h3>To the Editor.—</h3> Mosaic variants of Turner's syndrome have been reported to occur in about 20% of the cases and they show wide variations in their phenotypes.^1-4This report represents a case of Turner's syndrome with mosaic chromosomal abnormalities and unusual manifestations such as normal ovaries, multiple cardiac anomalies, and partial situs inversus. To our knowledge, the latter two manifestations have not been reported previously. <h3>Report of a Case.—</h3> An infant weighing 2,500 gm was born at 36 weeks gastation on Dec 19, 1969, to a 31-year-old gravida 7, para 2, white mother. Bradycardia (40 to 60 beats per minute) of the baby had been noted in prenatal examinations. Moderate cyanosis, hepatosplenomegaly, and a grade 4/6 systolic murmur were present at birth. The Apgar score was 5. The baby had low-set ears, a low hair line, a short and webbed neck, a shield chest with widely spaced nipples, and

Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease.

AT THE meeting of the Mid-Western Society for Pediatric Research in 1962, I reported on nine patients with remarkably similar facies with a previously unrecognized syndrome of valvular pulmonary stenosis and multiple extra cardiac anomalies.¹Review of the literature at that time showed that several males resembling these patients had been reported under several different headings, most commonly under the designation of, "the male Turner syndrome."^2-5There were no definite reports of similarly affected female patients. In that report I spoke in terms of a "new syndrome" in order to stress that this condition could affect not only males but also females, and that it was also clinically and etiologically different from the true Turner⁶syndrome. Since that time, a further eight patients, five males and three females, with valvular pulmonary stenosis and similar associated lesions have been studied. An additional two patients, one of each sex,

Incomplete differentiation of the cardiac valves: A report of 197 cases

Incomplete differentiation of cardiac valves is a relatively common, but infrequently recognized and documented congenital malformation of the cardiac valve involving single, multiple, or all cardiac valves. A total of 197 cases were reviewed. This group comprises 5 per cent of the gross congenital hearts examined at the Armed Forces Institute of Pathology over the past 15 years. In some cases, the only cardiac abnormality was the valvular lesion, but the great majority of cases presented other associated single or multiple congenital cardiac anomalies. The lesion is believed to be caused by varying degrees of incomplete development of the cardiac valves from their embryonic state. An implication of the significance of this entity is indicated by the finding of varying degrees of ischemic myocardial damage associated with aortic valve involvement. This complication is believed to be the result either of coronary hemodynamic disturbance or of local myocardial biochemical imbalance. Clinical awareness of incomplete differentiation of the valve and its possible complications should be stressed because of the obvious hazards of corrective operation in these hearts.

COREY AND I Study of an Infant with Multiple Cardiac Anomalies

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Corey and I: Study of an Infant with Multiple Cardiac Anomalies

Corey and I: Study of an Infant with Multiple Cardiac Anomalies

Cor biatriatum triloculare

A case of multiple cardiac anomalies—cor biatriatum triloculare—is herewith described which presented the following anatomical findings: 1. 1. Mitral atresia. 2. 2. Absent interventricular septum. 3. 3. Hypertrophied pulmonary artery becoming the descending aorta through a patent ductus arteriosus. 4. 4. Extreme hypoplasia of the aorta. 5. 5. Solitary (left) coronary artery. 6. 6. Bicuspid aortic valve. 7. 7. Accessory pulmonary vein leading from the upper portion of the right lung to the superior vena cava. 8. 8. Anomalous communication between the left auricle and the superior vena cava.