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In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) for long-term management are defined by both maximal and current coronary artery (CA) dimensions normalized as z-scores. We sought to determine the degree to which current recommended practice differs from past actual practice, highlighting areas for knowledge translation efforts. The International KD Registry (IKDR) included 1651 patients with CA aneurysms (z-score > 2.5) from 1999 to 2016. Patients were classified by AHA RL using maximum CA z-score (RL 3 = small, RL 4 = medium, RL 5 = large/giant) and subcategorized based on decreases over time. Medical management provided was compared to recommendations. Low-dose acetylsalicylic acid (ASA) use ranged from 86 (RL 3.1) to 95% (RL 5.1) for RLs where use was “indicated.” Dual antiplatelet therapy (ASA + clopidogrel) use ranged from 16% for RL 5.2 to 9% for RL 5.4. Recommended anticoagulation (warfarin or low molecular weight heparin) use was 65% for RL 5.1, while 12% were on triple therapy (anticoagulation + dual antiplatelet). Optional statin use ranged from 2 to 8% depending on RL. Optional beta-blocker use was 2–25% for RL 5, and 0–5% for RLs 3 and 4 where it is not recommended. Generally, past practice was consistent with the latest AHA guidelines, taking into account the flexible wording of recommendations based on the limited evidence, as well as unmeasured patient-specific factors. In addition to strengthening the overall evidence base, knowledge translation efforts may be needed to address variation in thromboprophylaxis management.

Anomalous origin of a coronary artery from the pulmonary artery (PA) is a rare congenital anomaly with the most common pattern being an anomalous left coronary artery (LCA) from the pulmonary artery (ALCAPA). A very rare pattern is the anomalous origin of a single coronary artery from the pulmonary artery (ASCAPA) wherein the single coronary ostium supplies both the right and left coronary systems with profound myocardial ischemia developing once PA pressures begin to fall after birth. Previous reports of this anomaly have all been based on post-mortem findings or pre-mortem angiographic diagnosis [1-3]. Notably, these children often present in extremis due to cardiogenic shock. Institution of veno-arterial extracorporeal life support can be catastrophic as it would lead to myocardial ischemia due to PA decompression. We present here the first echocardiographic-only diagnosis of ASCAPA in a 2-month-old child with the use of bubble contrast echocardiography to help confirm the diagnosis. The patient was resuscitated from a cardiac arrest on arrival with subsequent echocardiographic-only diagnosis. Surgical repair was undertaken with administration of cardioplegia into the pulmonary root with snaring of the branch PAs, and re-implantation of the single coronary to the aorta. At 3-month follow-up, he is thriving clinically with echocardiogram showing improving-though still somewhat depressed-left ventricular function.

BACKGROUND: Fetal aortic valvuloplasty (FAV) has shown promise in averting the progression of fetal aortic stenosis to hypoplastic left heart syndrome. Altered loading conditions due to valvar disease, intrinsic endomyocardial abnormalities, and procedures that alter endomyocardial mechanics may place patients with biventricular circulation (BiV) after FAV at risk for abnormal LV remodeling and function. METHODS: Using the most recent echo data on BiV patients after technically successful FAV (n=34) we evaluated LV remodeling pattern, risk factors for pathologic LV remodeling, and the association between LV remodeling pattern and LV function. RESULTS: Median age at follow up was 4.7 years (range 1.0–12.5). Cardiac interventions were common. At latest follow-up, no patient had hypoplastic LV. Nineteen patients (55%) had dilated LV and 5 (16%) patients had severely dilated LV. LV remodeling patterns were: 12 (35%) normal ventricle, 11 (32%) mixed hypertrophy, 8 (24%) eccentric hypertrophy or remodeling, and 3 (9%) concentric hypertrophy. Univariate factors associated with pathologic LV remodeling were long-standing AR, ≥ 2 cardiac interventions, EFE resection, and aortic or mitral regurgitation ≥ moderate at most recent follow-up. In multivariate analysis only long-standing AR fraction remained associated with pathologic remodeling. Pathologic LV remodeling was associated with depressed ejection fraction, lower septal E,’ and higher E/E’. CONCLUSION: Pathologic LV remodeling, primarily eccentric or mixed hypertrophy, is common in BiV patients after FAV and is related to LV loading conditions imposed by valvar disease. Pathologic remodeling is associated with both systolic and diastolic dysfunction in this population.

Congenital atrioventricular (AV) block is commonly associated with heterotaxy syndrome; together they have reportedly low survival rates (10–25 %). However, information about perinatal outcome and predictors of non-survival after prenatal diagnosis of this association is scarce. Therefore, we studied fetuses with heterotaxy syndrome and bradycardia or AV-block diagnosed between 1995 and 2011, and analyzed pre and post-natal variables. The primary outcome was death and the secondary outcome was pacemaker placement. Of the 154 fetuses with heterotaxy syndrome, 91 had polysplenia syndrome, 22/91(24 %) with bradycardia or AV-block. Thirteen (59 %) patients had sinus bradycardia at diagnosis, 8 (36 %) complete AV block, and 1 (5 %) second-degree AV-block. Three patients elected for termination of pregnancy (3/22, 14 %), 4 had spontaneous fetal demise (4/22, 18 %), and 15 (15/22, 68 %) were live-born. Of the fetuses with bradycardia/AV-block, 30 % presented with hydrops, 20 % had ventricular rates