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Our case report details the journey of a 16-year-old male patient with Shone complex and advanced heart failure. We highlight the pivotal role of the HeartWare Ventricular Assist Device (Medtronic) implantation in mitigating severe pulmonary hypertension, thereby facilitating his eligibility for a heart transplant. We discuss the subsequent management of post-transplant pulmonary hypertension and right ventricular dysfunction using targeted pulmonary vasodilators and inotropic support, underscoring the intricacies of postoperative care in pediatric heart transplant patients. This case emphasizes our observation of the critical role that left ventricular assist devices play in redefining transplant candidacy and the necessity for complex, ongoing management in pediatric heart transplant scenarios.

A parachute mitral valve (PMV) is a congenital mitral valve anomaly diagnosed in infancy, and it can also be discovered in adults during echocardiography. Surgical management is common in infants to prevent complications from left-heart obstructions. In adults, PMV may be found independently or with other cardiac defects. Prophylactic antibiotics are recommended for certain congenital heart anomalies before dental procedures. A study suggests reconsidering guidelines to include anomalies like bicuspid aortic valve and MVP for antibiotic prophylaxis. PMV, with transvalvular blood flow turbulence, may increase the risk of infective endocarditis, as seen in a reported case with a parachute-like mitral valve. Here, we present the case of a 62-year-old female incidentally found to have a PMV during an echocardiogram.

Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.

We aim to share the diagnostic methods and relevant findings of a rare case involving a 1-year-old girl with a variation of Shone syndrome, presenting with the coexistence of a single coronary artery and a fistula between the coronary artery and the right pulmonary artery. The patient had previously undergone aortic arch reconstruction surgery and during the preoperative evaluation, cardiac computed tomography angiography (CCTA) revealed the additional presence of a single coronary artery originating from the right, which fistulized into the right pulmonary artery distally. This finding was further confirmed by conventional angiography. This case report highlights the importance of CCTA in identifying and directing the treatment of additional anomalies that may accompany rare congenital abnormalities.

An 18-year-old primigravida woman underwent emergent percutaneous balloon stent repair of a severe coarctation of the aorta, allowing her to undergo an uneventful remaining pregnancy and the delivery of a healthy baby. Her case also demonstrates the association between maternal coarctation and fetal Shone complex. (Level of Difficulty: Beginner.).

We present a case of a 22-year-old male with dyspnea on exertion where computed tomography revealed complete Shone\'s complex. This case highlights the complementary role of computed tomography in the anatomical evaluation of patients with complex heart diseases.

Multilevel obstruction of left-sided heart structures was originally characterized by Shone et al. The formulation of an appropriate operative strategy remains challenging and needs to be individualized for this complex subset of patients. Intraoperative transesophageal echocardiography (TEE) not only helps in delineating spatial anatomy but also reveals associated anomalies that help in decision-making regarding operative strategies for these patients. Here, we discuss five such cases of Shone\'s anomaly presenting at varied age group with different associated anomaly in which intraoperative TEE played a pivotal role in the management.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. ALCAPA from the right pulmonary artery (RPA) is a small subset of this anomaly, with only a few reported cases to date. We describe an extremely uncommon association of Shone\'s complex with ALCAPA from the RPA in a case where simultaneous surgery for both anomalies was successfully carried out in a neonate.

Persistent fifth aortic arch is a rare congenital anomaly that can be discovered incidentally or at postmortem exam. It can be associated with major congenital heart malformations involving the systemic or the pulmonary circuits. It usually has no clinical significance but can be either, beneficial as in systemic outflow tract obstructions or cause hemodynamic compromise when associated with a significant left to right shunt. We report an infant with persistent fifth aortic arch associated with Shone\'s complex diagnosed accurately by transthoracic echocardiography and confirmed by cardiac catheterization and computed tomography.