Anomalous Left Coronary Artery Connected to the Pulmonary Artery in a 15-Year-Old Girl: Case Report and Discussion on Secondary Prevention of Sudden Death.

Background. Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is a rare congenital heart disease. Adaptive development of sufficient heterocoronary collaterality in the newborn may allow survival to a later age. In older children or adults, malignant ventricular arrhythmias can reveal the disease. Case Report. A 15-year-old girl was referred to the local hospital after a resuscitated out-of-hospital cardiac arrest. CT scan and coronary angiography revealed an ALCAPA. Direct aortic reimplantation of the left coronary artery was performed. Postoperative ECG monitoring showed short episodes of nonsustained ventricular tachycardia. Transthoracic echocardiography and cardiac MRI revealed subendocardial fibrosis of the anterolateral papillary muscle. Beta-blockade therapy was initiated at first intention. After hospital discharge, the patient reported several fainting without loss of consciousness. Considering sudden death nonrelated to effort, episodes of nonsustained ventricular tachycardia, and areas of myocardial fibrosis, the patient underwent subcutaneous cardioverter-defibrillator implantation. 6-month follow-up is satisfactory without clinical or rhythmic abnormalities. Discussion. Indication for surgical correction of ALCAPA is well defined, but rhythmic secondary prevention after resuscitated cardiac arrest is less consensual. Cardiac MRI is an essential tool in the identification of a potential rhythmic substrate and should be taken into account in the discussion of a preventive cardioverter-defibrillator implantation.

Predictive factors for residual hypertension following aortic coarctation stenting.

Native coarctation of the aorta (CoA) and recoarctation (reCoA) after initial surgical repair are frequently associated with hypertension (HT). Most CoA cases are amenable to transcatheter balloon angioplasty with stent implantation; however, the impact of stenting on arterial blood pressure (BP) is variable. We carried out a retrospective study to identify the predictive factors for residual HT despite optimal endovascular treatment. Patients who had undergone stent implantation for native CoA or reCoA with a pressure gradient of >20 mm Hg between the upper and lower limbs, between 2007 and 2015, were included. The geometry and level of hypoplasia of the aortic arch were determined by non-invasive imaging, and BP measurements were performed pre- and post-procedure. Thirty consecutive patients (median age: 18.5 years; 76.7% male) were included. Twenty-three patients had HT before the procedure and 14 (46.7%) had post-procedural HT despite optimal endovascular treatment. Residual HT post-stenting was associated with longer stent length and gothic arch geometry. Age and body mass index (BMI) were also associated with residual HT. The pathologic association of abnormal arch geometry and aortic stent placement may lead to a loss of aortic compliance that is further increased by high BMI and older age. Determination of a patient's aortic arch anatomy and clinical profile can assist in defining those at high risk of residual HT despite optimized isthmic stent implantation.

Speckle-tracking imaging in patients with Eisenmenger syndrome.

BACKGROUND: Adults with Eisenmenger syndrome have a survival advantage over those with idiopathic pulmonary arterial hypertension. Improved survival may result from preservation of right ventricular (RV) function. AIMS: To assess left ventricular (LV) and RV remodelling in patients with Eisenmenger syndrome compared to a control population, using speckle-tracking imaging. METHODS: Adults with Eisenmenger syndrome and healthy controls were enrolled into this prospective two-centre study. Patients with Eisenmenger syndrome with low acoustic windows, irregular heart rhythm or complex congenital heart disease were excluded. Clinical assessment, B-type natriuretic peptide (BNP), 6-minute walk test and echocardiography (including dedicated views to perform offline two-dimensional-speckle-tracking analysis) were performed on inclusion. RESULTS: Our patient population (n=37; mean age 42.3 ± 17 years) was mostly composed of patients with ventricular septal defect (37.8%) or atrial septal defect (35.1%). Compared with the control population (n=30), patients with Eisenmenger syndrome had reduced global LV longitudinal strain (-17.4 ± 3.5 vs. -22.4 ± 2.3; P<0.001), RV free-wall longitudinal strain (-15.0 ± 4.7 vs. -29.9 ± 6.8; P<0.001) and RV transverse strain (25.8 ± 25.0 vs. 44.5 ± 15.1; P<0.001). Patients with Eisenmenger syndrome also more frequently presented a predominant apical longitudinal and transverse strain profile. Among patients with Eisenmenger syndrome, those with a post-tricuspid shunt presented with reduced global LV longitudinal strain but increased RV transverse strain, compared to patients with pre-tricuspid shunt. CONCLUSION: Patients with Eisenmenger syndrome had impaired longitudinal RV and LV strain, but present a relatively important apical deformation. RV and LV remodelling, as assessed by speckle-tracking imaging, differ between patients with pre- and post-tricuspid shunt.