Background-Accurate diagnosis of major aortopulmonary collaterals (MAPCAs) and partial anomalous pulmonary venous drainage (PAPVD) in adult patients with congenital heart disease is important but problematic. Threedimensional contrast-enhanced magnetic resonance angiography (MRA) provides a minimally invasive technique to allow detailed studies in a single breath-hold. Methods and Results-We assessed the role of contrast-enhanced 3D MRA in 29 consecutive adult patients with a diagnosis of MAPCAs (nϭ16) or PAPVD (nϭ13) made by echocardiogram, cardiac catheterization, or surgical inspection. MRA was performed with a 3D spoiled gradient-echo technique with intravenous gadolinium-DTPA (0.2 mmol/kg). In both types of pathology, there was excellent correlation between MRA and the cardiac catheterization, echocardiogram, or surgical inspection. Additional information was gained for patients with MAPCAs on confluence and size of pulmonary arteries (nϭ13 had central arteries), pulmonary artery stenosis (nϭ3), aneurysmal dilatation of pulmonary artery (nϭ1), and additional anomalous vascular abnormality (nϭ3). Shunt assessment, where present (9 of 16), showed patency in all cases (100%). For adults with PAPVD, further information was obtained on drainage origin (nϭ11). There were no complications. Conclusions-Contrast-enhanced 3D MRA provides a fast, noninvasive, radiation-free method of accurate and comprehensive diagnosis of MAPCAs and PAPVD in adult patients.
The standard treatment of coarctation of the aorta is surgical. In the last 2 decades, however, treatment by catheter intervention has become more widespread, using either balloon angioplasty or primary stent implantation. Balloon angioplasty was originally used for recurrent coarctation after surgical repair but has now been shown equally effective for unoperated coarctation. The procedure produces a satisfactory gradient reduction in approximately 80% of patients, with transverse arch hypoplasia the main predictor of poorer outcome. Rates of restenosis and aneurysm formation are less than 10%. Primary stent implantation has been suggested as an option potentially superior to angioplasty alone. Stent implantation limits elastic recoil and potentially reduces aneurysm formation by reducing the amount of balloon stretch required. The incidence of suboptimal gradient reduction is low, probably 5% or less, as is the rate of restenosis. Aneurysm formation, vascular complications, and stent migration also occur in less than 5%. Catheter interventions are now an established treatment strategy for coarctation, with a good success rate and safety profile. The outcome for native and recurrent coarctation appears similar. The authors believe that for most adult patients with coarctation of the aorta, catheter intervention should be offered as initial therapy.
Adult patients with congenital heart disease provide a wide variety of challenges for the interventional cardiologist. Procedures can broadly be divided into dilatation or closure. The most common interventions in our own practice are closure of atrial septal defects and patent foramen ovale, although closure of postoperative interatrial communications in Mustard, Senning, or Fontan patients is also possible. Transcatheter patent ductus arteriosus closure is also now routine, and occlusion of coronary artery fistulae can be safely attempted using coil embolisation. Balloon dilatation of pulmonary valve stenosis has excellent success rates, and dilatation or stenting of pulmonary artery stenoses is also beneficial in selected cases. Although aortic valve dilatation in adults has mixed results, dilatation or stenting of aortic coarctation is now becoming more widespread, and has promising results. Dilatation procedures may also be applicable to the postoperative patient with conduit or baffle stenosis.
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