P. Syamasundar Rao

This review addresses the diagnosis and management of ventricular septal defects (VSDs). The VSDs are classified on the basis of their size, their number, and their location in the ventricular septum. Natural history of VSDs includes spontaneous closure, development of pulmonary hypertension, onset of infundibular obstruction, and progression to aortic insufficiency. While initial diagnostic approaches such as careful history-taking, physical examination, chest X-rays, and electrocardiograms provide basic information, echo-Doppler studies are essential for assessing the defect's clinical significance and determining the need for intervention. Intervention is usually indicated for symptomatic patients with moderate- to large-sized VSDs. Surgical closure is advised for perimembranous, supracristal and inlet VSDs, and for deficits involving prolapsed aortic valve leaflets. While percutaneous methods to occlude perimembranous VSDs with Amplatzer Membranous VSD Occluder are feasible, they are not recommended due to a notable risk of inducing complete heart block in a significant number of patients. Alternatively, percutaneous and hybrid methods employing the Amplatzer Muscular VSD Occluder are effective for treating large muscular VSDs. The majority of treatment options have demonstrated satisfactory outcomes. However, practitioners are urged to exercise caution in managing small defects to avoid unnecessary procedures and to ensure timely intervention for large VSDs to prevent pulmonary vascular obstructive disease.

Patent ductus arteriosus (PDA) is a congenital cardiac defect (CCD) and comprises 8% to 10% of all CCDs. Following the description of surgical closure by Gross and colleagues in the 1930s, it has become a standard mode of therapy and remained so until the description of transcatheter techniques to occlude PDA by Porstmann, Rashkind and their associates in the late 1960s. This review paper discusses transcatheter occlusion techniques with buttoned devices, Giantuco coils , and Amplatzer devices with particular attention to author’s contributions to these methods.

Balloon aortic valvuloplasty (BAV) effectively decreases peak pressure gradients through the aortic valve both at the time of the procedure and during follow-up. This paper presents the author’s observations on the utility of BAV in treating congenital aortic valve stenosis (AVS). Previous work by the author noted intermediate-term restenosis and late-onset aortic insufficiency (AI). Factors contributing to restenosis include age under 3 years and post-balloon residual aortic valve gradients exceeding 30 mmHg. Repeat balloon valvuloplasty has been found to address restenosis effectively. However, the onset of AI at late follow-up is a significant disadvantage of BAV. Despite this, BAV is currently considered a beneficial treatment option for managing AVS in children, adolescents, and young adults. In contrast, seniors with calcific AVS do not experience relief from obstruction with BAV and are candidates for aortic valve replacement through transcatheter methodology, a procedure not reviewed in this paper. BAV provides relief of obstruction across the aortic valve and functions as an alternative to surgical intervention. Consequently, BAV is considered a preferred choice in addressing aortic stenosis in the pediatric patient.

Open access peer-reviewed chapter

Transcatheter interventions that are useful in the management of valvar aortic stenosis will be reviewed. This chapter focuses on congenital aortic valve stenosis. The procedure of balloon aortic valvuloplasty (BAV) and the results were reviewed; BAV offers good relief of aortic valve obstruction and serves as substitute to surgery and is considered a favored option in the management of aortic stenosis in all age groups. However, BAV in elderly patients with calcific aortic stenosis offers only a temporary relief of aortic valve obstruction and BAV is not recommended for this subgroup of patients. Except for neonates, most patients are discharged home within 24-hours after BAV. While there is conclusive data for provision of pressure gradient relief both acutely and at follow-up as well as deferral of any surgery after BAV, the development of aortic insufficiency (AI) at long-term follow-up is a most important drawback. In neonates, severe AI may develop necessitating surgical intervention. Notwithstanding these drawbacks, BAV is presently believed to be a therapeutic procedure of option in the treatment of valvar aortic stenosis in pediatric and young adult patients. Methodical follow-up to identify reappearance of aortic obstruction and development of substantial AI is suggested.

Balloon valvuloplasty/angioplasty techniques have been available to address valvar and vascular obstructions associated with congenital heart defects (CHDs) since the 1980s. The objective of this paper is to provide a pictorial rendition of the author’s observations over the last four decades on these techniques. In this paper, balloon pulmonary valvuloplasty (BPV) for treating isolated pulmonary stenosis (PS), PS associated with cyanotic CHDs, and bioprosthetic valve in the pulmonary position were reviewed. Balloon dilatation leads to a decrease in the peak systolic pressure gradient through the pulmonary valve during BPV, as well as at intermediate-term and long-term evaluation. Problems, such as the reappearance of obstruction at intermediate-term follow-up and pulmonary insufficiency (PI) at long-term follow-up, have been documented in isolated PS cases but are infrequent. Repeat balloon valvuloplasty for restenosis has been successfully used. The development of infundibular obstruction in PS cases was also reviewed. To decrease the frequency and degree of PI, the author has revised the recommendations for balloon/annulus ratios used for BPV from the previous 1.2 – 1.4 to 1.2 – 1.25. In patients with PS associated with cyanotic CHD, improvement of oxygen saturations at the time of BPV and enhanced anatomy during follow-up were observed. BPV of bioprosthetic valves results in minimal improvement in the pulmonary valve gradient, and stents may be a better alternative to address this problem.

Congenital heart defects (CHDs) are structural abnormalities of the heart or blood vessels that occur while cardiac structures are being formed in utero