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When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Cleveland Clinic Journal of Medicine. 2010 November;77(11):821-828 | 10.3949/ccjm.77a.09172
November 1, 2010|Cleveland Clinic Journal of Medicine
David Fox, ME, PA-C;Ganesh P. Devendra, BA;Stephen A. Hart, BS;Richard A. Krasuski, MD
Author and Disclosure Information

David Fox, ME, PA-C
Department of Cardiovascular Medicine, Section of Clinical Cardiology, Cleveland Clinic

Ganesh P. Devendra, BA
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Education Institute, Cleveland Clinic

Stephen A. Hart, BS
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Education Institute, Cleveland Clinic

Richard A. Krasuski, MD
Department of Cardiovascular Medicine, Section of Clinical Cardiology, Cleveland Clinic

Address: Richard A. Krasuski, MD, Department of Cardiovascular Medicine, Section of Clinical Cardiology, J2-4, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; krasusr@ccf.org

ABSTRACTMost babies born with tetralogy of Fallot undergo corrective surgery and survive to adulthood. However, as they get older they are prone to a number of long-term problems, and they often do not receive expert-level follow-up care. This review of the adult complications of tetralogy of Fallot should help primary care practitioners identify these patients, make appropriate and timely referrals, and educate patients and their families.

KEY POINTS

  • The major long-term complication of tetralogy of Fallot repair is pulmonary valve insufficiency, which leads to right heart failure. Other problems include atrial and ventricular arrhythmias and sudden cardiac death.
  • Surgical pulmonary valve replacement is the standard of care, but the optimal time to do this is unclear.
  • Novel and experimental therapies include percutaneous pulmonary valve replacement and medical therapy with pulmonary arterial vasodilators.

NOVEL THERAPIES

Percutaneous valve replacement

The future of pulmonary valve replacement may lie in percutaneous procedures.

Figure 4. The Melody transcatheter pulmonary valve (Medtronic; Minneapolis, MN). Left, pulmonary angiography helps guide catheter delivery of the valve into the right ventricular outflow tract. The angiogram also demonstrates severe pulmonary valve insufficiency. Right, a fully expanded valve (inset), with angiography demonstrating near-complete resolution of pulmonary valve insufficiency.
The Melody transcatheter pulmonary valve (Medtronic, Minneapolis, MN) is implanted percutaneously via the femoral vein, guided by fluoroscopy (Figure 4). Early results appear very promising, with significant reductions in pulmonary insufficiency and right ventricular size, as well as improved exercise tolerance.58–60

The Melody valve is now approved through a humanitarian device exemption (ie, based on demonstrated safety without proven efficacy) for patients who have a prior pulmonary conduit now complicated by either stenosis or regurgitation.

If percutaneous pulmonary valve replacement proves to have reasonable long-term durability, it has the potential to dramatically shift the balance toward earlier intervention.

Pulmonary vasodilator drugs

Our group is examining whether pharmacologic therapy can alter the clinical outcome in patients with pulmonary insufficiency (due to either tetralogy of Fallot or valvotomy done to treat remote pulmonary stenosis). Specifically, we are using MRI to examine the effects of inhaled nitric oxide, a selective pulmonary vasodilator. Preliminary results suggest that such a strategy may work, and we are designing a trial to examine the longer-term benefit of using an oral drug with similar properties.

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