Aortic valve prolapse and aortic regurgitation in patients with ventricular septal defect.

Aortic valve prolapse (AVP) was detected in 82 (7.5%) of 1096 patients with ventricular septal defect (VSD) (in 50 at initial echocardiographic examination and in 32 at follow-up) by echocardiography. Of 82 patients with AVP, aortic regurgitation (AR) was detected in 53 (65%) (in 28 at initial echocardiographic examination and in 25 at follow-up), resulting in an incidence of AR of 4.8% (of VSD). The percentage of AVP (20.8%) and AR (16.7%) in muscular outlet VSDs was larger than the percentage of AVP (10.6%) and AR (6.8%) in perimembranous VSDs (p <0.05). Fourty-four patients were followed medically after AVP appeared (3 months to 10.8 years; median, 2.1 years). Initially, there was no AR in 24 of these patients, trivial AR in 7, and mild AR in 13. Trivial AR developed in 6 (25%) and mild AR developed in 3 (13%) of 24 patients who had no AR (in 5 of them within 1 year and in 9 of them within 2 years). In 2 (29%) of 7 patients, trivial AR progressed to mild AR during a median of 2 years, and in 4 (31%) of 13 patients, mild AR progressed to moderate AR during a median of 1.1 years. We recommend frequent echocardiographic evaluation (every 6 months) for detecting of appearance of AR in patients with perimembranous or muscular outlet VSD after AVP develops and for evaluating the progression of AR in patients with perimembranous or muscular outlet VSD, AVP, and trivial AR. In addition, we recommend surgical intervention in patients with perimembranous or muscular outlet VSD, AVP, and mild AR because of rapid progression of mild AR to moderate AR.

Evolution of ventricular septal defect with special reference to spontaneous closure rate, subaortic ridge and aortic valve prolapse.

The medical records of 685 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. Patients had been followed for a mean of 3 +/- 2.5 years and median 2.2 years by echocardiography. VSD was perimembranous in 65.7% (450), trabecular muscular in 30.8% (211), muscular outlet in 2.3% (16), muscular inlet in 0.7% (5), and doubly committed subarterial in 0.5% (3). Defect size was classified in 76% (517) as small, in 18% (124) as moderate, and in 6% (44) as large. VSD closed spontaneously in 27% (186 of 685 patients) by ages 40 days to 13.7 years (mean, 2.1 +/- 2.2 years; median, 1.3 years). Sixty-six of 450 perimembranous defects (15%) and 120 of 211 trabecular muscular defects (57%) closed spontaneously (p <0.001). Defect size became small in 15% of patients with VSD at mean 2.9 +/- 2.3 years (median, 2.3 years). Aneurysmal transformation was detected in 56% (254), left ventricular-to-right atrial shunt in 8.4% (38), subaortic ridge in 5.8% (26), aortic valve prolapse in 11.7% (53), and aortic regurgitation in 7.3% (33) of 450 patients with perimembranous defect. There was no statistical significance between the age at closure and the age of detection of aneurysmal transformation in the patients with perimembranous defect (p = 0.25).

Spontaneous regression of huge cardiac rhabdomyoma in an infant.

Rhabdomyomas are the most common primary cardiac tumors in childhood, and are often associated with tuberous sclerosis. We report a huge rhabdomyoma in an asymptomatic four hour old infant who presented initially with a murmur due to moderate subaortic stenosis. Followup showed regression of the tumour. Although the indications for surgical resection of symptomatic tumors are well established, medical follow-up should be the prefered treatment.

Regression of right ventricular pressure in ventricular septal defect in infancy: a longitudinal color-flow Doppler echocardiographic study.

To determine the course of right ventricular pressure (RVP) in patients with isolated ventricular septal defect (VSD) and factors influencing it, unselected 148 infants were followed-up longitudinally with color-Doppler echocardiography from a median age of 1 month for 201 patient-years. The patients were divided into three groups by absolute echographic size of VSD: group I, 4 to 7 mm. Sixty percent belonged to group I. Muscular defects dominated in group I, perimembranous defects dominated in group II, and those with outlet extensions dominated in group III. Peak systolic RVP was obtained by Doppler-estimated difference between systolic brachial artery and peak gradient across the VSD. Initial RVP ranged between 15 and 95 mmHg and increased in parallel to the size of defect. According to the regression equations RVP decreased in general by 0.17 mmHg per month. This correlated significantly with the size of the defect. In group I, the rate of decrease was very fast and is best expressed by a log function of time (r = -0.67, r(2) = 0.45). In groups II and III the rate of decrease was less steep and had a greater variability. RVP normalized in 100% in those of group I and in 90% of group II, at median ages of 0.17 and 0.33 years, respectively. Median Q(p):Q(s) values were 1.5, 2.2, and 3.0 in groups I-III, respectively. The outcome depended on the size of VSD. Spontaneous closure was observed in 51% of group I, 10% of group II, and none of group III. The rate was higher in muscular defects. Congestive heart failure was present in 53% and 100% in groups II and III, respectively. Death rate was 2.03%, all in patients with large defects. It is concluded that the temporal course of RVP with time can be estimated fairly well by the regression equation presented in relation to the initial size of the VSD.

Regression of right ventricular dominance in the electrocardiogram after birth: a longitudinal follow-up of a healthy cohort from birth to 3 years.

To establish the incidence and course of right ventricular (RV) conduction delay during the regression of right ventricular dominance, 12 serial ECGs were recorded at regular intervals until age 36 months in a cohort of 43 normal neonates. Additionally, ECGs were recorded similarly in 6 infants from the age of 1 month and in 7 from 2 months. The right precordial R waves were classified according to their amplitudes and their occurrence before or after the S wave. QR duration and its ratio to QRS were measured in right and left chest leads. The incidence of notched R waves, which is very rare in neonates, increased after the first month and occurred before the peak of R. Thereafter, the height of the notch decreased and then occurred after the peak of R. This turned into an r' (after the S wave) and finally the amplitude of r' decreased further and evolved into a notched S pattern. An rSR' or qRS pattern in the right precordial leads was never observed. An abnormal incomplete right bundle branch block during infancy can be distinguished from physiological right ventricular conduction delay by the unvarying amplitude of the R' wave, preceded by an S wave.

The natural course of isolated ventricular septal defect during adolescence.

Serial changes in patients with isolated VSD during adolescence have not previously been investigated. Hemodynamic status, diameter of the defect, and growth were studied yearly in 106 children with VSD. The mean duration of the follow-up was 13.16 years and ranged in 80% of subjects from 7 to 19 years (1395 patients years). The mean ages at pre- and postpuberty were 8.62 and 16.67, respectively. The presented longitudinal study, in which losses due to death and operation were minimal (4%), ideally reflected the natural history of VSD. Although weight showed retardation during prepuberty, this lag was caught up by the end of adolescence. Stature showed no retardation in pre- and postpuberty. Cardiothoracic ratio decreased significantly from a mean of 0.48 to 0.44 and showed normal variation. Although the mean defect diameter at prepuberty was 5.33 mm, this decreased to 2.7 postpubertally. The individual decrease (1.7 +/- 2.34 mm) was significant (t = 5.349, p < 1/10(5)). The defect closed spontaneously in 24 (22.6%). In the 75 patients without pulmonary hypertension and with mild left-to-right shunting, 52 remained in the same class and spontaneous closure was observed in 23. In the 24 patients with moderate to severe left-to-right shunt, this decreased in 23 and only one remained stable. The 2 patients (1.9%) with Eisenmenger syndrome remained stable and 1 died. Aortic regurgitation developed in 10 patients (9.4%); however, this was of mild degree in most of them. No infective endocarditis was observed. It is concluded that patients with VSD should be followed closely through adolescence, because the diameter of the defect, as well as left-to-right shunting, can decrease, and it is concluded that the spontaneous closure of the defect is to be expected in a considerable 23%, and aortic prolapse or mild regurgitation may develop in approximately 10%.

Long-term prognosis of acute rheumatic carditis with combined aortic and mitral regurgitation.

Thirty-one patients with acute rheumatic carditis associated with aortic (AR) and mitral regurgitation (MR) who received corticosteroid anti-inflammatory therapy for an average of nine weeks were followed up for 332 patient-years (mean: 10.71 years). The age of onset was 9.34 (1 SD = +/- 1.94) years. The patients were classified according to degree of left ventricular volume overloading (LVH), duration of pre-treatment interval and regularity of penicillin prophylaxis. The probability of the yearly disappearance of MR, AR and both lesions were calculated for the total group and in relation to affecting subgroups. The mean yearly rate of disappearance of AR was 2 percent after a 10 percent probability in the first year. This rate was 6.83 percent per year for MR, and it increased to 10.15 percent per year in the patients in whom therapy was initiated in less than three weeks, and decreased to 3.14 percent in the patients in whom therapy was initiated after three weeks. The disappearance of both AR and MR was observed in only two patients (about 1% per year); MS developed in two patients (6.45%) and the mortality rate was 3.2 percent in 332 patient-years (0.0001/year).

Long-term prognosis of rheumatic mitral regurgitation: presentation of yearly prognostic regressions in relation to affecting factors.

Acute rheumatic isolated mitral regurgitation was followed up in 86 children for 730 patient-years. The grade of severity of regurgitation was classified according to degree of left ventricular enlargement, which was based on an original method of correction of cardiothoracic ratio on the chest x-ray, taking into consideration the diaphragmatic level, and electrocardiographic criteria for age. A combination of both quantitative estimations, after the regression of a detectable pericardial effusion reduced false-positive radiologic and false-negative electrocardiographic results. Linear regressions of percentage of yearly disappearance of MR were presented in relation to the grades of severity; the mean yearly rate of disappearance of 5.8% increased to 9.08% in mild, and decreased to 1.84% in severe regurgitation. The effect of other beneficial factors such as the shortness of the pretreatment interval and regularity of prophylaxis could be demonstrated by keeping the grade of severity of regurgitation constant. Thus, chances for recovery in reported years after onset, up to ten years, can readily be calculated from the presented regressions with regard to the appropriate subgroups.