Recurrence of Congenital Heart Disease in Cases with Familial Risk Screened Prenatally by Echocardiography

Objectives. To evaluate the recurrence of congenital heart disease (CHD) in pregnant women with familial risk who had been referred for fetal echocardiography. Material and Methods. 1634 pregnancies from 1483 women with familial history of CHD in one or more relatives were studied. Fetal cardiologic diagnosis was compared with postnatal findings at 6 months or at autopsy. Results. Total recurrence rate of CHD was 3.98%, 4.06% in single familial risk, 2.9% in double, and 5% in multiple risk. It was 3.5% in case of one previously affected child; 4.5% with 2 children; 5.2% with the mother alone affected and 7,5% with father alone affected and 3.5% with a single distant relative. Exact concordance of CHD was found in 21.5% and a partial concordance in 20% of cases. Conclusions. Our data show a higher recurrence rate of CHD than previously published data and high relative risk ratios compared to normal population

Transcatheter Closure of Perimembranous Ventricular Septal Defects

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Transcatheter closure of atrial septal defect in young children Results and follow-up

AbstractObjectivesThis study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure of secundum atrial septal defect (ASD) in young children.BackgroundResults of ASD transcatheter closure in adults are widely reported but there are no large published series concerning young children.MethodsBetween December 1996 and February 2002, 48 of 553 patients percutaneously treated at our institution were children age ≤5 years. Indications for closure were: elective closure in 32 patients; frequent respiratory infections in 8; failure to thrive in 2; liver transplantation in 5; and a fenestrated Fontan in 1. The procedure was carried out under general anesthesia with fluoroscopy and transesophageal control. Two different devices were used: 1) the CardioSEAL/StarFLEX (CS/SF) and 2) the Amplatzer septal occluder (ASO). Basal physical examinations and echocardiograms were performed prior to the procedure and at follow-ups (1, 6, and 12 months, and yearly thereafter).ResultsThe mean age at closure was 3.6 ± 1.3 years. A CS/SF was used in 10 subjects; an ASO was used in 38 patients. No deaths or immediate major complications occurred. The total occlusion rate was 87% at procedure, rising to 94% at discharge. The mean follow-up was 18 ± 14 months. No midterm major or minor complications occurred. The occlusion rate rose to 100% at 12 months of follow-up. Symptomatic patients improved significantly.ConclusionsIn the current era and in experienced hands, ASD closure can be performed safely and successfully, even in very young children

Echocardiographic Assessment after Surgical Repair of Tetralogy of Fallot

Surgical correction of Tetralogy of Fallot is still one of the most frequently performed intervention in pediatric cardiac surgery, and in many cases it is far from being a complete and definitive correction. It is rather an excellent palliation that solves the problem of cyanosis but predisposes the patients to medical and surgical complications during follow-up. The decision-making process regarding the treatment of late sequel are among the most discussed topics in adult congenital cardiology. In postoperative Fallot patients, echocardiography is used as the first method of diagnostic imaging and currently allows both a qualitative observation of the anatomical alterations and a detailed quantification of right ventricular volumes and function, of the right ventricular outflow tract, and of the pulmonary valve and pulmonary arteries. The literature introduced many quantitative echocardiographic criteria useful for the understanding of the pathophysiological mechanisms involving the right ventricle and those have made much more objective any decision-making processes

Regression of Cardiac Rhabdomyomas Producing a Severe Aortic Stenosis: Case Report and Discussion of the Literature

We describe a case of a voluminous rhabdomyoma (R) detected by fetal echocardiography at 32 weeks’ gestation (w.g.) obstructing the left ventricular inflow and aortic outflow tract, with a moderate aortic gradient at birth, not needing immediate surgery. At follow-up, the mass progressively regressed, leaving the aortic valve partly damaged, with a gradient that increased to a maximum of 100 mmHg at 9 years. The girl was then operated on successfully by a plasty of the aortic valve. The literature regarding R is discussed