eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Ventricular Septal Defect, General Concepts: Follow-up

Author: Prema Ramaswamy, MD, Assistant Professor of Pediatrics, Mount Sinai School of Medicine of New York University; Adjunct Assistant Clinical Professor of Pediatrics, St George's University School of Medicine; Co-Director of Pediatric Cardiology, Maimonides Medical Center, Lutheran Medical Center, and Coney Island Hospital
Coauthor(s): Patturajah Anbumani, MD, MBBS, MS, MCh, Associate Medical Director, Best Medical Care; Former Associate Medical Director, Jeanes Hospital, Temple University Health System; Former Adjunct Clinical Assistant Professor, New York College of Osteopathic Medicine; Former Clinical Assistant Professor, Department of Medicine, State University of New York-Downstate; Kuruchi Srinivasan, MD, Consulting Staff, Department of Internal Medicine, Nazareth Hospital
Contributor Information and Disclosures

Updated: Feb 10, 2009

Follow-up

Further Outpatient Care

• Even patients with small ventricular septal defects (VSDs) require indefinite follow-up.
• Patients with perimembranous ventricular septal defects who have undergone aneurysmal closure have a high incidence of left ventricular (LV)-to-right atrial (RA) shunts and a 6% incidence of subaortic ridge as shown in a large Chinese study.⁶
• Emphasize the importance of good dental hygiene and prophylactic antibiotics for infective endocarditis.
• With increasing age, the incidence of aortic leaflet prolapse and aortic insufficiency increases in children with the doubly committed and perimembranous type of ventricular septal defect.

Complications

• Eisenmenger complex
  • Eisenmenger complex is the most severe complication.
  • Fixed and irreversible pulmonary hypertension develops, resulting in reversal of the left-to-right shunt to a right-to-left shunt.
• Secondary aortic insufficiency
  • Secondary aortic insufficiency is associated with prolapse of aortic valve leaflets.
  • This is rare in children younger than 2 years.
  • This complication is observed only in 5% of patients with ventricular septal defect.
  • The incidence is higher in supracristal ventricular septal defects than in perimembranous ventricular septal defects.
• Aortic regurgitation
  • The development of aortic regurgitation in association with doubly committed subarterial ventricular septal defect is a well-known phenomenon.
  • Aortic regurgitation is due to a poorly supported right coronary cusp combined with the Venturi effect produced by the ventricular septal defect jet, resulting in cusp prolapse.
• RV outflow tract obstruction
  • Right ventricular (RV) outflow tract obstruction was noted in 7% of a large cohort of ventricular septal defect in France.¹⁸  The investigators noted the obstruction to be infundibular.
  • A later angiocardiographic study showed that the obstruction was most often secondary to anomalous muscle bundles and only rarely infundibular.¹⁹
• Subaortic obstruction
  • Discrete fibrous subaortic stenosis is occasionally associated with a ventricular septal defect.
  • This complication is most often reported with perimembranous ventricular septal defects and can first appear after either spontaneous or surgical closure.
  • Zielinsky et al concluded that anterior or posterior malalignment of the outlet or the conal septum is present in all patients with a ventricular septal defect who develop discrete subaortic stenosis.²⁰
• Infective endocarditis
  • Infective endocarditis is rare in children younger than 2 years.
  • In the presence of infective carditis in pulmonary circulation, meticulously record the patient's history and echocardiographically investigate the left-to-right shunt. In ventricular septal defects, both the systemic and pulmonary circulation may be affected; hence, vegetation manifests on both sides.
  • Embolization is highly expected despite the morphology of the vegetation. In general, vegetation more than 10 mm, particularly if pedunculated, should be regarded as an indication for surgical intervention, even in the absence of symptoms.
  • Infection is usually located at the ridge of the ventricular septal defect itself or the tricuspid leaflet.

Prognosis

• The current surgical mortality rate is less than 2% for isolated ventricular septal defects.
• Children with small ventricular septal defects are asymptomatic and have excellent long-term prognoses.
• The outcome of medical therapy for children with moderate or large ventricular septal defects is as follows:
  • Many infants improve, showing evidence of a gradual decrease in the magnitude of the left-to-right shunt when aged 6-24 months. Carefully assessing the cause of the decrease in left-to-right flow, which can reflect an increase in pulmonary vascular resistance (PVR), a decrease in the relative size of the defect, or the development of RV outflow tract hypertrophy, resulting in functional or anatomic obstruction.
  • Most children with ventricular septal defect remain in stable condition or improve after infancy. Heart failure rarely occurs after infancy. Anemia, respiratory infection, endocarditis, or the development of an associated lesion (eg, aortic insufficiency) can trigger a recurrence of symptoms.
• A few patients who develop severe pulmonary vascular obstructive disease with predominant right-to-left shunts (Eisenmenger syndrome) at the time of referral require symptomatic therapy.
  • Cyanosis progressively increases, and exercise capacity decreases.
  • RBC reduction by means of partial-exchange transfusion may relieve symptoms associated with extreme polycythemia (eg, headache, extreme fatigue).
  • Select patients may be candidates for lung or heart-lung transplantation.

Patient Education

• Lifestyle changes (ie, exercise before and after surgery or catheterization) may not be required.
  • A restrictive ventricular septal defect with a functional normal heart imposes no exercise limitation.
  • Although patients can safely participate in competitive sports without restriction, adults in this category are uncommon. An important exception is the adult whose moderately restrictive perimembranous ventricular septal defect decreased in size or closed spontaneously in infancy. However, 2-dimensional echocardiography with Doppler interrogation and color flow imaging should be performed to determine whether the defect closed by means of formation of a septal aneurysm.
• Unrestricted exercise after surgical closure of a moderate-to-large ventricular septal defect is permitted if the following criteria are met:
  • Postoperative PA pressure
  • Absence of clinically significant disturbances in ventricular rhythm during maximal exercise stress testing and during 24-hour ambulatory electrocardiography
  • Two-dimensional echocardiographic evidence of an intact ventricular septum with normalization of LV and LA size and LV function
  • Twelve-lead scalar ECG revealing little or no evidence of LV volume overload or RV pressure overload
• For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education articles Tetralogy of Fallot and Ventricular Septal Defect.

Miscellaneous

Medicolegal Pitfalls

• Failure to distinguish pathologic murmur of ventricular septal defect (VSD) from innocent physiologic murmurs
• Failure to recognize subtle signs and symptoms of ventricular septal defect
• Failure to detect ventricular septal defect early and to refer patient for surgical repair to prevent complications, including the onset of pulmonary vascular disease

Special Concerns

• Pregnancy and prenatal care
  • The presence or lack of early care is not a factor in congenital cardiovascular malformations (CCVMs).
  • A major objective of medical management is to minimize the factors that interfere with the limited circulatory reserve of pregnant women with ventricular septal defects.
  • Because anxiety is a special concern in a primigravida, the expectant mother should be prepared mentally for pregnancy, labor, delivery, and puerperium.
  • Diuretics can be used judiciously to manage edema of cardiac failure, but they should not be used to treat edema of normal pregnancy.
  • Pregnant women with heart disease should limit themselves to moderate isotonic exercise.
  • Maternal mortality in pregnant women with heart disease has been associated with the functional class.
  • Ventricular septal defect associated with pulmonary vascular disease is one of the 2 major maternal cardiac risks; the other is pulmonary edema.
• Labor and delivery
  • In women with functionally mild unoperated lesions and in patients after successful surgical repair, management of labor and delivery is the same as for pregnant women without a ventricular septal defect.
  • The recommendations of the American Heart Association state that no antibiotic prophylaxis is required for a normal vaginal delivery.
  • For pregnant women with functionally important congenital cardiac disease (unoperated or operated), the management of labor, delivery, and the puerperium is crucial to minimize risk.
  • Induced vaginal delivery is preferred over cesarean delivery. Cesarean delivery results in twice the blood loss of vaginal delivery. In addition, it is associated with risks of wound infection, uterine infection, thrombophlebitis, and potential postoperative complications.
• Effect of CCVM on society
  • Cardiovascular malformations are the leading cause of premature mortality from congenital anomalies.
  • The progressive reduction in the mortality rate over the past 20 years reflects the major advances in the management of heart defects in infants.
  • Premature mortality computed as years of potential life lost before age 65 years is a way of measuring the long-term effect of heart defects.
  • Three defects rank among the most frequent causes of premature death due to congenital malformations; ventricular septal defect is one of them. The other 2 are hypoplastic left heart and transposition of the great arteries.
  • The direct and indirect costs are great. The economic cost to society remains difficult to delineate. For each infant or child who dies from complications of a ventricular septal defect, society is denied the fruit of his or her labor.
• Points to remember
  • After a bicuspid aortic valve, ventricular septal defect is the most common congenital heart defect.
  • The axiom "the louder the murmur, the smaller the defect" does not always apply.
  • The murmurs heard in early infancy, which disappear by age 1 year, probably represent spontaneous closure of the defects.
  • Perimembranous defects account for 80% of all ventricular septal defects.
  • The recognition of the diastolic murmur of aortic insufficiency, in the presence of classic findings of ventricular septal defect, should make the diagnosis of supracristal variety likely.
  • The supracristal ventricular septal defect most commonly occurs in Asian patients.
  • The supracristal ventricular septal defect is also known as a doubly committed ventricular septal defect.
  • Elevated pulmonary resistance may be maintained in some patients despite therapy directed at the ventricular septal defect and may, in fact, represent a primary disease of the pulmonary vessels.
  • The defects observed in adulthood are usually small or medium sized because the vast majority of patients with isolated large defects come to medical and often surgical attention early in life.
  • An experienced pediatric cardiologist can accurately assess newly referred patients with murmurs on clinical examination with a sensitivity of 96% and a specificity of 95%.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthors Ashmitha Srinivasan, BA; Viswanath Natesan, MD; and Sharmila Srinivasan, BS, to the development and writing of this article.

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