We present two cases (females aged 23 and 36 years) with newly diagnosed systemic lupus erythematosus who developed lupus myocarditis concurrent with lupus nephritis. Both exhibited acute decompensated heart failure with dyspnea, edema, and cardiomegaly. Common key findings included strongly positive ANA blot, nephrotic-range proteinuria, acute kidney injury, and markedly elevated NT-proBNP (>35,000 pg/mL). Transthoracic echocardiography confirmed biventricular dysfunction (left ventricular ejection fraction [LVEF]: 20%-35% initially), moderate-to-severe tricuspid regurgitation, moderate-to-severe pulmonary arterial hypertension, and mild pericardial effusions. Both received high-dose pulse dexamethasone (40-60 mg IV), hydroxychloroquine (HCQ), diuretics, beta-blockers, SGLT2 inhibitors, and HCQ. The fulminant case additionally underwent plasma exchange. Both the patients, over the next few weeks, received cyclophosphamide therapy and achieved LVEF recovery to 50%-55% within 1-2 months, with improved renal function and resolution of heart failure symptoms. These cases underscore the reversibility of lupus myocarditis with early aggressive immunosuppression.

Racemose hemangioma of the bronchial artery is a rare vascular malformation characterized by abnormal communication between the bronchial and pulmonary vasculature. Coronary arterial inflow may further complicate minimally invasive cardiac surgery because excessive collateral flow can impair myocardial protection and increase intracardiac return during cardiac arrest. We report a case of atrial functional mitral regurgitation and tricuspid regurgitation associated with racemose bronchial artery malformation with coronary arterial inflow from the left circumflex artery. Preoperative embolization of systemic feeders from the bronchial and left subclavian arteries was followed by minimally invasive valve repair and surgical ligation of the coronary inflow vessel. The postoperative course was uneventful. A staged hybrid strategy may be effective for managing racemose bronchial artery malformation with coronary arterial inflow.

Quantification of the regurgitation fraction (RF) in pediatric patients requires cardiac magnetic resonance imaging (CMR). Three-dimensional echocardiography (3DE) can be used to measure right and left ventricular volumes and therefore has the potential to calculate the RF by comparison of stroke volumes. 3DE volume acquisitions can be acquired in only a few heartbeats, often as part of a standard pediatric study. This study compared the quantification of single valve regurgitation by 3DE with CMR, the latter serving as the gold standard. Patients < 18 years of age were included in the study if they had mild regurgitation of one valve and a biventricular circulation. Patients were excluded if they had more than mild regurgitation of another valve, or a significant shunt lesion. Full right and left ventricular volume data sets were obtained using a Philips -iE33 or EPIQ CVx machine (X5 or X7 ultrasound probe) as a part of studies done for clinical reasons. 3DE volumes and RFs were analyzed retrospectively by blinded echocardiographers using 4D Cardioview (Tomtec Imaging Systems) and compared with ventricular volumes and RF obtained by CMR. Additionally, 3DE-derived ventricular volume ratios (VVR) were analyzed for correlation and agreement with CMR. Inter- and intra-observer reliability were assessed for 3DE ventricular volumes and RF. 43 patients were included with 7 patients having two separate paired 3DE and CMR studies. The median patient age was 8.4 (IQR; 3.6-12.0) years. Anesthesia, for the CMR study, was used for 20 (40%) of the paired studies. Valve involvement was: mitral-18 (33%), tricuspid-12 (22%), pulmonary-5 (9%), and aortic-20 (36%). Agreement between 3DE and CMR ventricular volumes as well as 3DE and CMR VVR was excellent, with good agreement for the regurgitation fraction across the two modalities. 3DE compared well with CMR in delineation of severity of regurgitation (mild, moderate, and severe). Intra- and inter-observer reliability was excellent for ventricular volumes, VVR, and RF. Quantitative assessment of single valve regurgitation using 3DE compared well with CMR in pediatric patients. Ventricular volume ratios obtained using 3DE strongly correlated with CMR. The short time for image acquisition, lower cost, and relative ease of acquisition should allow for application of 3DE to quantify single valve regurgitation in this population and will be useful for interpretation of serial studies.