Baffle leaks are a frequently encountered and often overlooked complication after the atrial switch procedure for transposition of the great arteries. Baffle leaks are present in up to 50% of non... Show moreBaffle leaks are a frequently encountered and often overlooked complication after the atrial switch procedure for transposition of the great arteries. Baffle leaks are present in up to 50% of non-selected patients, and while they initially may not cause clear symptoms, they can complicate the hemodynamic course and influence the prognosis in this complex patient group. A shunt from the pulmonary venous atrium (PVA) to the systemic venous atrium (SVA) can lead to pulmonary overflow and subpulmonary left ventricular (LV) volume overload, while a shunt from the SVA to the PVA can result in (exercise-associated) cyanosis and paradoxical embolism. We report three cases of baffle leaks in patients with systemic right ventricular (sRV) failure late after the atrial switch procedure. Two symptomatic patients who presented with exercise-associated cyanosis due to SVA to PVA shunting over the baffle leak underwent successful percutaneous baffle leak closure with a septal occluder device. One patient with overt sRV failure and signs of subpulmonary LV volume overload due to PVA to SVA shunting was managed conservatively, as baffle leak closure was expected to lead to an increase in sRV end-diastolic pressure and aggravation of sRV dysfunction. These three cases illustrate the considerations made, challenges faced, and necessity of a patient-tailored approach when addressing baffle leaks. Show less
Verheijen, D.B.H.; Stoeger, J.L.; Kley, F. van der; Schalij, M.J.; Jongbloed, M.R.M.; Vliegen, H.W.; ... ; Egorova, A.D. 2022
Coarctation of the aorta (CoA) is a congenital heart defect that is associated with a bicuspid aortic valve (BAV), ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic... Show moreCoarctation of the aorta (CoA) is a congenital heart defect that is associated with a bicuspid aortic valve (BAV), ascending aorta dilatation, intracerebral aneurysms, and premature atherosclerotic disease. The first presentation during late adulthood is rare and is frequently driven by late sequelae. Hypertrophic collateral arteries can develop aneurysms which are at risk for spontaneous rupture, however, treatment recommendations for these aneurysms are scarce. Here, we describe the clinical course and percutaneous treatment strategy of a patient with a late diagnosis of a pin-point CoA, a BAV with moderate regurgitation, and an exceptionally large aneurysm of a collateral artery. A 59-year-old woman was diagnosed with Streptococcus bovis endocarditis of a BAV with moderate aortic valve regurgitation and small vegetation (<5 mm) on the non-coronary cusp. Work-up revealed hypertension and adenocarcinoma in situ of the ascending colon, considered the bacteremia porte d'entree, for which a curative hemicolectomy was performed. Echocardiography showed a narrowing of the aorta distal from the origin of the left subclavian artery with the antegrade diastolic flow with a pathognomonic "sawtooth" pattern and an estimated pressure gradient of >70 mmHg. Computed tomography angiography (CTA) showed a network of well-developed collateral arteries and a levoatriocardinal vein. One of the collateral arteries arising from the left subclavian artery revealed an exceptionally large aneurysmatic dilation (29 x 24 x 24 mm). The invasive assessment confirmed a hemodynamically significant CoA. Treatment involved balloon dilatation and placement of a covered stent at the site of the pin-point CoA and a percutaneous coronary intervention (PCI) of the stenosis in the left anterior descending artery. No residual gradient over the CoA was observed. Antihypertensive drugs could be discontinued, and CTA performed 4 months later showed regression and thrombosis of the numerous collaterals and, importantly, thrombosis of the large aneurysm. This case illustrates the late diagnosis of CoA with associated congenital heart defects and late sequelae including hypertension, BAV endocarditis, coronary artery disease, and aneurysm formation of the extensive collateral network. The patient underwent pharmacological and percutaneous treatment, ultimately resulting in the alleviation of the CoA, normalization of the blood pressure, reduction of collateral flow, and thrombosis of the large aneurysm of the collateral artery. Show less
The American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology... Show moreThe American Heart Association and American College of Cardiology published practice guidelines for the management of adult congenital heart disease in 2018 and the European Society of Cardiology published analogous guidelines in 2020. Although there are broad areas of consensus between the 2 documents, there are important differences that impact patient management. This review discusses key areas of agreement and disagreement between the 2 guidelines, with discussion of possible reasons for disagreement and potential implications. (J Am Coll Cardiol 2021;78:1904-1918) (c) 2021 by the American College of Cardiology Foundation. Show less
