Chronological T-wave alternation before and after the onset of arrhythmogenic right ventricular cardiomyopathy.

Identification of arrhythmogenic right ventricular cardiomyopathy (ARVC) during childhood is challenging due to the lack of specific ECG manifestation. We report chronological ECG alteration before several years of the ARVC onset in two affected children. Their ECG at the age of 6 years was almost normal for their age, and their chronological ECGs exhibited inversion of T wave in inferior leads, which are typical for ARVC, developed at younger age than that in precordial leads. In addition, the leftmost T-wave inversion in the precordial lead shifted toward the left in our patients, which is a sharp contrast to its physiological transition.

Effect of Minocycline Pleurodesis in Infants With Refractory Chylothorax After Palliative Surgery for Complex Congenital Heart Disease.

Chylothorax is a critical complication after surgery for congenital heart disease, which markedly compromises the postoperative course with increased mortality. As the cardiovascular load additively causes stagnation of the thoracic duct, chylothorax after palliative cardiac surgery can be highly refractory to the therapies. Here we report a case of two patients with refractory chylothorax attributed to hemodynamic load which was successfully treated with minocycline pleurodesis. In combination with congenital heart disease, extremely low birth weight coupled with prematurity in case 1 and venous obstruction with excessive volume load due to additional aortopulmonary shunt in case 2 additively increased resistance to the therapies, including fasting with total parenteral nutrition (TPN), XIII factor supplementation, octreotide infusion, as well as the use of steroids. As pleural effusion was sustained at more than 50 ml/kg/day, the condition of both patients deteriorated severely; pleurodesis using minocycline was urgently introduced. Pleural effusion declined at every session and both cases were in remission in a few sessions without unfavorable acute reaction. No symptoms suspecting chronic adverse effects were observed during follow-up, including respiratory dysfunction, pulmonary hypertension, tooth staining, or abnormal bone mineralization. Although the application of minocycline for children should be minimized, minocycline pleurodesis can be an option for patients with refractory and life-threatening chylothorax.

Reliability of respiratory-triggered two-dimensional cine k-adaptive-t-autocalibrating reconstruction for Cartesian sampling for the assessment of biventricular volume and function in patients with repaired tetralogy of Fallot.

OBJECTIVE: To compare left ventricular (LV) and right ventricular (RV) volume, function, and image quality of a respiratory-triggered two-dimensional (2D)-cine k-adaptive-t-autocalibrating reconstruction for Cartesian sampling (2D kat-ARC) with those of the standard reference, namely, breath-hold 2D balanced steady-state free precession (2D SSFP), in patients with repaired tetralogy of Fallot (TOF). METHODS: 30 patients (14 males, mean age 32.2 ± 13.9 years) underwent cardiac magnetic resonance, and 2D kat-ARC and 2D SSFP images were acquired on short-axis view. Biventricular end-diastolic volume (EDV) and end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF), and LV mass (LVM) were analysed. RESULTS: The 2D kat-ARC had significantly shorter scan time (35.2 ± 9.1 s vs 80.4 ± 16.7 s; p < 0.0001). Despite an analysis of image quality showed significant impairment using 2D kat-ARC compared to 2D SSFP cine (p < 0.0001), the two sequences demonstrated no significant difference in terms of biventricular EDV, LVESV, LVSV, LVEF, and LVM. However, the RVESV was overestimated for 2D kat-ARC compared with that for 2D SSFP (73.8 ± 43.2 ml vs 70.3 ± 44.5 ml, p = 0.0002) and the RVSV and RVEF were underestimated (RVSV = 46.2±20.5 ml vs 49.4 ± 20.4 ml, p = 0.0024; RVEF = 40.2±12.7% vs. 43.5±14.0%, p = 0.0002). CONCLUSION: Respiratory-triggered 2D kat-ARC cine is a reliable technique that could be used in the evaluation of LV volumes and function. ADVANCES IN KNOWLEDGE: 2D cine kat-ARC is a reliable technique for the assessment LV volume and function in patients with repaired TOF.

Transcatheter Coil Embolization of Single Coronary Artery Fistula Using the Occlusion Test.

The case of a patient in whom hemodynamic and electrocardiographic studies using the occlusion test for coronary artery fistulas (CAF) were safely performed prior to catheter embolization is reported. A 1-year-old girl had a separate right coronary artery arising from a left single coronary artery that formed a significant coronary artery fistula to the right ventricle. Coronary steal by the large coronary artery fistula narrowed the left coronary artery. The right coronary artery branches could not be clearly identified due to an overlap with the fistula. Due to the long porous CAF, embolic procedures could cause serious complications. We confirmed the safety by performing an occlusion test of the CAF's proximal blood vessels. Following total occlusion of the CAF for 10 minutes, pulmonary arterial pressure and aortic blood pressure were not significantly changed. No bradycardia, atrioventricular block, or ST changes were observed. Coil embolization treatment was performed safely. For patients with long distal CAF complicated with a single coronary artery, myocardial ischemia and conduction system disorders can be identified by performing the occlusion test before embolization.

A refractory hypertensive patient with fibromuscular dysplasia and multicystic dysplastic kidney.

The case of a 16-year-old male patient with left renal artery stenosis due to fibromuscular dysplasia (FMD) and an atrophic kidney due to a right multicystic dysplastic kidney (MCDK) who presented with refractory hypertension is reported. On continuous Doppler imaging, the peak systolic velocity (PSV) at the stenotic site of the left renal artery was 404 cm/s. The FMD formed a "string-of-beads" appearance on computed tomographic angiography (CTA) and renal artery angiography. Percutaneous transluminal renal angioplasty (PTRA) with a balloon catheter was performed, after which the left renal artery returned to near normal, and his blood pressure decreased to within the normal range. In this case, the main cause of refractory hypertension was considered to have been renal artery stenosis due to left FMD.