Supracristal Ventricular Septal Defect Treatment & Management

Updated: Feb 03, 2021
  • Author: Ira H Gessner, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Approach Considerations

Once the diagnosis of supracristal ventricular septal defect (VSD) has been made, there should be careful follow-up for the development of aortic valve insufficiency. This necessitates not only periodic physical examination with auscultation but also serial echocardiograms, because these diagnostic studies are more sensitive than auscultation in detecting aortic valve regurgitation.

Because spontaneous closure is uncommon in supracristal VSDs and aortic valve insufficiency is common and surgical closure is recommended in most cases. Aortic valve insufficiency in supracristal VSD is usually progressive and warrants an aggressive approach with early intervention to avoid aortic valve deformity and replacement.

Aortic valve insufficiency caused by supracristal VSD must be differentiated from that caused by an abnormal aortic valve (usually a bicuspid valve). Surgical intervention is usually delayed in the latter disorder, because the abnormal aortic valve typically requires replacement rather than repair in cases of aortic valve insufficiency.


Surgical Treatment

Because of the orientation of the right ventricular outflow tract, a surgical approach from the right atrium may not allow adequate visualization of the ventricular septal defect (VSD). [20, 21] Incision into the main pulmonary artery, which exposes the defect through the pulmonic valve, has proved successful.

Repair may be achieved with patch or suture closure, depending on the size of the defect. Aortic valvuloplasty is often, but not always, necessary, and incision through the aortic root can allow adequate visualization for valve repair (Trusler technique). The approach through the main pulmonary artery avoids the need for incision into the right ventricle. Care should be taken to avoid capturing the aortic cusp into one of the patch sutures. [22, 23]

Intraoperative transesophageal echocardiographic monitoring before and after cardiopulmonary bypass can be extremely helpful in precisely defining aortic valve prolapse and the severity of valve insufficiency, which determine the necessity of valvuloplasty. [24]

More extensive damage to the aortic valve from long-standing prolapse and distortion may require valve replacement.

Follow-up care after supracristal VSD repair and aortic valvuloplasty is essential to ensure that the aortic insufficiency has been corrected completely.

Shamsuddin et al examined the outcomes of surgical closure for supracristal VSDs in 17 patients with a median age of 6 years (range: 2 to 9 years). After surgery, 2 patients (12%) had trivial residual shunting; aortic valvular regurgitation (AoVR) persisted in 6 patients (35%), reducing to trivial in 5 patients (29%) and mild in 1 patient (6%). The mean stay in the intensive care unit was 2.6 ± 1.2 days; the mean stay in the hospital was 6.8 ± 0.8 days; and the mean follow-up was 14 ± 4 months, with no early or late deaths and without clinical deterioration. [25]

Gan et al found evidence that percutaneous perventricular device closure of supracristal VSDs is safe and efficacious, with acceptable short-term outcomes. In their study, percutaneous perventricular device closure was successful in 15 out of 16 patients (93.8%) with supracristal VSDs. No deaths, residual shunting, new valve regurgitation, or arrhythmias occurred perioperatively or during the short-term follow-up period, and the mean hospital stay was 3.5 ± 2.0 days. [26] Thirty-eight patients with supracristal VSD were added during the follow-up period (n=54). No death, residual shunt, new valve regurgitation, or arrhythmia was reported during the mid-term& (2.5 years) follow-up period. The procedure was successful in 53 (98.1%) cases. One patient in the original group developed pericardial effusion and tamponade. [27]



Activity level is determined by the age at which signs or symptoms develop. Infants with large left-to-right shunts, particularly with complex left heart obstruction, will present soon after birth with congestive heart failure symptoms of poor feeding, diaphoresis, and tachypnea. Patients with small left-to-right shunts without aortic valve insufficiency or with only trivial aortic valve insufficiency generally should be allowed full activity without restriction.

Older patients with more significant aortic valve insufficiency should be restricted from competitive athletics and from sustained isometric types of activities (eg, weightlifting, rope pulls, sustained heavy lifting on the job).