Comments: The clinical presentation of patients with TGA/VSD/PS is varied and depends both on the severity and site of pulmonary stenosis and on the size and location of the VSD. If the cyanosis is severe, a systemic-to-PA shunt, combined with atrial septectomy, is often required in early life. Occasionally, patients are well balanced, as with our case here, allowing for a more definitive repair later on in life.

The Rastelli operation is the preferred surgical route for patients with TGA/VSD/PS. The VSD is used to make the LVOT; the Rastelli procedure includes placement of a VSD baffle (Dacron or Gore-Tex), directing blood from the LV to the aorta via the VSD. Surgical enlargement of the VSD may be necessary. The pulmonary trunk is often transected, and the pulmonary valve or the root of the pulmonary trunk is oversewn. A conduit is inserted from an incision made in the RV to the distal part of the pulmonary trunk. This operation was first described in 1969 and advantageously leaves the morphologic LV as the systemic ventricle. The aortic valve overrides the ventricular septum. This feature and the pulmonary stenosis relate to the posterior deviation of the outlet septum. Pulmonary stenosis, in turn, with a malaligned VSD maintains LV pressure equivalent to the RV that supports the systemic circulation. Morphologically speaking, this malformation is to some extent analogous to TOF in the setting of discordant ventriculoarterial connection. Late complications of the Rastelli procedure include conduit obstruction/regurgitation, LVOT obstruction, and arrhythmias.

Prosthetic valve endocarditis (PVE) is an endovascular infection occurring on parts of a prosthetic valve or on reconstructed native heart valves. It has a low but increasing frequency ranging from 0.1% to 2.3% per patient year. PVE occurs either perioperatively (early PVE) or more than a year after surgery as community-acquired (late PVE). These two forms usually involve different microorganisms. Infective endocarditis accounts for 4% of admissions to a specialized ACHD unit. Reparative surgery does not prevent endocarditis except for closure of a VSD and a patent ductus arteriosus. LVOT lesions are most commonly involved.

Conduit stenosis after the Rastelli procedure is very common. Most patients will require multiple conduit replacements, as the longevity of currently used conduits is often only a few years in children and perhaps 10 to 20 years in older patients. Because of homograft unavailability and problems with early homograft preservation, the use of Hancock bioprosthetic valved conduits was favored before the 1980s. They consist of a standard unstented porcine aortic valve, sutured into the center of a woven fabric conduit (Dacron). Later cryopreservation improved the preservation of homografts, which then became the conduit of choice in some centers, while heterograft valved conduits continued to be preferred in other centers.

Substantial hemodynamic changes occur during pregnancy, and pregnancy may unmask previously asymptomatic problems. The 30% to 50% increase in cardiac output may result in maternal arrhythmia and/or heart failure, as well as potential fetal problems. The high-risk group includes women with prosthetic valves, conduits, and obstructive cardiac lesions, as may be seen after the Rastelli operation.

Nearly all homografts eventually require replacement. Factors that adversely affect graft longevity include a higher systolic gradient immediately after placement, younger age, longer donor ischemic time, smaller homograft size, extracardiac location, or the use of an aortic homograft in an older patient.

Echocardiography often has difficulty evaluating such valved conduits, and other modalities, such as MRI or heart catheterization, may be needed.

Arrhythmia is the most common reason for the hospitalization of adults with congenital heart disease. There is a high incidence of late arrhythmias, both supraventricular and ventricular, which are generally felt to be associated with chamber dilatation, hypertrophy, and/or ventricular dysfunction. Scar tissue, complex tunnel repair, and severely stenosed conduits are additional risk factors. Correction of residual hemodynamic abnormalities may be the most important tool in the treatment of arrhythmia.

Comments: Dyspnea in a patient with complex congenital heart disease can be secondary to many factors, some of which are mechanical and/or reversible and should be investigated. Beyond these, it is becoming increasingly recognized that late ventricular dysfunction is a common cause of dyspnea.

Comments: The parasternal lift reflects the raised RV pressure. The second heart sound is single because only the substernal aortic component can be heard. The long systolic murmur is related to severe conduit obstruction. The diastolic murmur reflects prosthetic conduit regurgitation.