Efficacy of Drug-Coated Balloon Angioplasty in Pulmonary Vein Stenosis or Total Occlusion.

BACKGROUND: Current therapies for pulmonary vein stenosis (PVS) or pulmonary vein total occlusion (PVTO) involving angioplasty and stenting are hindered by high rates of restenosis. OBJECTIVES: This study compares a novel approach of drug-coated balloon (DCB) angioplasty and stenting with the current standard of care in PVS or PVTO due to pulmonary vein isolation (PVI). METHODS: A retrospective single-center study analyzed patients with PVS or PVTO due to PVI who underwent either angioplasty and stenting (NoDCB group; December 2012-December 2016) or DCB angioplasty and stenting (DCB group; January 2018-January 2021). Multivariable Andersen-Gill regression analysis assessed the risk of restenosis and target lesion revascularization (TLR). RESULTS: The NoDCB group comprised 58 patients and 89 veins, with a longer median follow-up of 35 months, whereas the DCB group included 26 patients and 33 veins, with a median follow-up of 11 months. The DCB group exhibited more PVTO (NoDCB: 12.3%; DCB: 42.4%; P = 0.0001), with a smaller reference vessel size (NoDCB: 10.2 mm; DCB: 8.4 mm; P = 0.0004). Follow-up computed tomography was performed in 82% of NoDCB and 85% of DCB, revealing lower unadjusted rates of restenosis (NoDCB: 26%; DCB: 14.3%) and TLR (NoDCB: 34.2%; DCB: 10.7%) in the DCB group. DCB use was associated with a significantly lower risk of restenosis and TLR (HR: 0.003: CI: 0.00009-0.118; P = 0.002). CONCLUSIONS: The novel approach of DCB angioplasty followed by stenting is effective and safe and significantly reduces the risk of restenosis and reintervention compared with the standard of care in PVS or PVTO due to PVI.

Lifetime Management of Adolescents and Young Adults with Congenital Aortic Valve Disease.

In this invited expert review, we focus on evolving lifetime management strategies for adolescents and young adults with congenital aortic valve disease, acknowledging that these patients often require multiple interventions during their lifetime. Our goal is to preserve the native aortic valve when feasible. Leveraging advanced multimodality imaging, a detailed assessment of the aortic valve and root complex can be obtained, and a surgically approach tailored to an individual patient's anatomy and pathology can be used. In turn, aortic valve repair and reconstruction can be offered to a greater number of patients, either as a definitive strategy or as a component of a staged strategy to delay the need for aortic valve replacement until later in life when more options are available.

Transcatheter management of obstructed baffle repairs of partial anomalous pulmonary veins: a case series.

Background: Partial anomalous pulmonary venous return (PAPVR) can be surgically corrected using a pericardial baffle. This baffle can become obstructed post-surgery, leading to pulmonary hypertension and right heart dysfunction if not detected and corrected. Case summary: We describe three patients with occluded PAPVR baffles who underwent drug-coated balloon angioplasty and stenting of the obstructed baffle. In each case, baffle obstruction was detected post-operatively on surveillance cross-sectional imaging, and an invasively measured pulmonary capillary wedge-to-left atrium gradient was noted to be elevated. Post-intervention, each patient had an improvement in baffle flow by angiography as well as lung perfusion as assessed by nuclear medicine scintigraphy. Discussion: Given the subtle symptomatology of obstructed PAPVR pericardial baffle repairs, surveillance imaging is necessary to detect occluded baffles and intervene before downstream right heart disease and pulmonary hypertension develops. Given the high rates of re-stenosis in pulmonary vein stenting, pre-treatment of occluded PAPVR baffles with drug-coated balloons may help reduce re-intervention rates.

Caring for the Critically Ill Adult Congenital Heart Disease Patient.

PURPOSE OF REVIEW: This review aims to discuss the unique challenges that adult congenital heart disease (ACHD) patients present in the intensive care unit. RECENT FINDINGS: Recent studies suggest that ACHD patients make up an increasing number of ICU admissions, and that their care greatly improves in centers with specialized ACHD care. Common reasons for admission include arrhythmia, hemorrhage, heart failure, and pulmonary disease. It is critical that the modern intensivist understand not only the congenital anatomy and subsequent repairs an ACHD patient has undergone, but also how that anatomy can predispose the patient to critical illness. Additionally, intensivists should rely on a multidisciplinary team, which includes an ACHD specialist, in the care of these patients.

Anomalous Aortic Origin of a Coronary Artery.

BACKGROUND: Although anomalous aortic origin of a coronary artery (AAOCA) is associated with risk of sudden cardiac arrest (SCA), there is a spectrum of disease, with the appropriate management for many remaining unclear. Increasing data warrant review for an updated perspective on management. METHODS: A panel of congenital cardiac surgeons, cardiologists, and imaging practitioners reviewed the current literature related to AAOCA and its management. Survey of relevant publications from 2010 to the present in PubMed was performed. RESULTS: The prevalence of AAOCA is 0.4% to 0.8%. Anomalous left coronary artery is 3 to 8 times less common than anomalous right coronary, but carries a much higher risk of SCA. Nevertheless, anomalous right coronary is not completely benign; 10% demonstrate ischemia, and it remains an important cause of SCA. Decision-making regarding which patients should be recommended for surgical intervention includes determining anatomic features associated with ischemia, evidence of ischemia on provocative testing, and concerning cardiovascular symptoms. Ischemia testing continues to prove challenging with low sensitivity and specificity, but the utility of new modalities is an active area of research. Surgical interventions focus on creating an unobstructed path for blood flow and choosing the appropriate surgical technique given the anatomy to accomplish this. Nontrivial morbidity has been reported with surgery, including new-onset ischemia. CONCLUSIONS: A proportion of patients with AAOCA demonstrate features and ischemia that warrant surgical intervention. Continued work remains to improve the ability to detect inducible ischemia, to risk stratify these patients, and to provide guidance in terms of which patients warrant surgical intervention.

Transcatheter Aortic Valve Replacement in Congenital Heart Disease.

Transcatheter aortic valve replacement is not widely used in patients with congenital heart disease. We describe our single-center experience of transcatheter aortic valve replacement in congenital heart disease, demonstrating short-term feasibility and safety, role in lifetime management of congenital aortic valve disease, and use as a bridge to recovery, future surgery, or transplantation.