Prevalence of congenital heart disease

The heart in higher vertebrates develops from a simple tube into a complex organ with four chambers specialized for efficient pumping at pressure. During this period, there is a concomitant change in the level of myocardial organization. One important event is the emergence of trabeculations in the luminal layers of the ventricles, a feature which enables the myocardium to increase its mass in the absence of any discrete coronary circulation. In subsequent development, this trabecular layer becomes solidified in its deeper part, thus increasing the compact component of the ventricular myocardium. The remaining layer adjacent to the ventricular lumen retains its trabeculations, with patterns which are both ventricle- and species-specific. During ontogenesis, the compact layer is initially only a few cells thick, but gradually develops a multilayered spiral architecture. A similar process can be charted in the atrial myocardium, where the luminal trabeculations become the pectinate muscles. Their extent then provides the best guide for distinguishing intrinsically the morphologically right from the left atrium. We review the variations of these processes during the development of the human heart and hearts from commonly used laboratory species (chick, mouse, and rat). Comparison with hearts from lower vertebrates is also provided. Despite some variations, such as the final pattern of papillary or pectinate muscles, the hearts observe the same biomechanical rules, and thus share many common points. The functional importance of myocardial organization is demonstrated by lethality of mouse mutants with perturbed myocardial architecture. We conclude that experimental studies uncovering the rules of myocardial assembly are relevant for the full understanding of development of the human heart.

https://onlinelibrary.wiley.com/doi/pdf/10.1002/%28SICI%291097-0185%2820000401%29258%3A4%3C319%3A%3AAID-AR1%3E3.0.CO%3B2-O

Developmental patterning of the myocardium.

Ventricular septal defects are the most common congenital heart defect. They vary greatly in location, clinical presentation, associated lesions, and natural history. The present article describes the clinical aspects of ventricular septal defects and current management strategies.

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Ventricular Septal Defects

Aim To report the experience of 23 tertiary referral European Centres on transcatheter closure of congenital ventricular septal defects (VSD).

Methods and results Implantation of transcatheter devices was attempted in 430 patients (pts) with congenital VSDs until July 2005. The following anatomic types were present: 119 muscular, 250 perimembranous, 16 multiple, 45 residual post-surgery. Median VSD size was 7 mm (range 3–22), fluoroscopy time 33 min (range 3–146). Devices implanted were Amplatzer muscular or membranous devices in 364, PDA devices in 12, ASD devices in seven, Starflex in seven, and coils in nine patients. Procedure was successful in 410 cases (95%). Complications: device embolization in five cases (surgery in two, catheter retrieval in three), aortic regurgitation in 14 cases (two of which requiring surgery), tricuspid regurgitation in 27 cases (no surgery was necessary), minor rhythm disturbances in 10 pts, death in one patient, complete heart block (cAVB) in 16 pts [perimembranous 12 of 250 (5%), muscular one of 119 (0.8%), residual post-surgery VSD three of 45 (6.7%)]. CAVB was transient in six patients, requiring permanent pace-makers in 10 cases (3.8%) (six early, four late). In the multivariate analysis, the only variable associated with a risk of the occurrence of complication was age ( P = 0.012) and weight ( P = 0.0035). In the univariate analysis, risk factors for the development of cAVB were, device type ( P = 0.03) and VSD location ( P = 0.05). After the multivariable Cox proportional hazards analysis, no risk factor was found.

Conclusion Transcatheter closure of congenital VSDs offers encouraging results. Complications are limited; the most relevant one seems to be the device related to cAVB in perimembranous VSD. More experience and long-term follow-up are mandatory to assess safety and effectiveness of this procedure as an alternative to conventional surgery.

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Transcatheter closure of congenital ventricular septal defects: results of the European Registry

Transcatheter closure of perimembranous ventricular septal defects: early and long-term results.

Ventricular septal defects (VSDs) are the most common congenital heart malformations seen in children. Because spontaneous closure occurs frequently, patients with small VSDs should be followed clinically with no limitations except endocarditis prophylaxis. Surgical closure is recommended for only small defects with significant associated lesions such as aortic regurgitation, aortic valve prolapse, right or left ventricular outflow obstruction, tricuspid regurgitation, left ventricle to right atrial shunt, or recurrent endocarditis. Enlarging left ventricular size or deteriorating left ventricular function would also be an indication for surgical repair. Moderate and large VSDs in infancy often require treatment of congestive heart failure with diuretics, digitalis, and afterload reduction. Surgical closure before 9 months of age is indicated for large VSDs and by 2 years of age for moderate shunts to prevent pulmonary vascular obstructive disease and the consequences of long-standing volume overload. Device closure of VSD is still in the investigational stage but holds promise for treatment of apical or multiple muscular VSDs.

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de Moor M

Papers

Ventricular Septal Defect.