Infant congenital heart disease prevalence and mortality in French Guiana: a population-based study.

Background: Few studies have assessed the prevalence and mortality of simple or complex congenital heart diseases (CHD) in newborns. In Latin America and Caribbean (LAC), CHD epidemiology seems highly variable, with few population-based assessments and different methodologies between studies. To date, the situation in French Guiana, a French overseas territory located in South America between Brazil and Suriname, has never been described. Methods: We analysed CHD prevalence, characteristics and related infant mortality in French Guiana, with a population-based registry analysis of all fetal and live birth CHD cases in infants under 1 year (January 2012-December 2016). Findings: Overall, 33,796 births (32,975 live births) were registered, with 231 CHD (56 fetuses), including 215 live births. Most frequent CHD categories were anomalies of the ventricular outflow tract and extra-pericardial trunks, and ventricular septal defects. 18.6% (43/231) chromosomal or genetic anomalies, and 6.5% (15/231) terminations of pregnancy were observed. Total CHD prevalence was 68.4 [95% CI: 67.9-68.8] per 10,000, while live birth prevalence was 65.2 [95% CI: 64.7-65.7] per 10,000. Total infant mortality was 9.4/10,000 live births [95% CI 9.1-9.7], with highest rates for functionally univentricular hearts (FUH). Interpretation: A distinct profile for CHD is highlighted in French Guiana with elevated mortality linked to FUH. A potential determinant of the recognized excess mortality risk might be the presence of chromosomal or genetic anomalies in about a fifth of all CHD. This helps us to better understand CHD burden in this part of South America and provides future keys towards reducing CHD-related infant mortality. Funding: The authors received no financial support for the present research, authorship, and/or publication of this article.

Giant Localized Atheroma Mimicking a Coarctation of the Aorta: A Rare Entity to Consider.

We describe the case of a patient who developed resistant hypertension due to a giant atheroma with acquired physiologic mimic of coarctation of the aorta. This presentation illustrates an extremely rare etiology to consider in adults in whom aortic isthmus stenosis remains often of congenital origin. (Level of Difficulty: Intermediate.).

A case report of mycotic pseudoaneurysm in childhood: an unusual complication of coarctation of the aorta.

BACKGROUND: We report on an unusual case of a 3 year-old girl with coarctation of the aorta complicated by mycotic pseudoaneurysm and infected with Streptococcus pneumoniae. CASE SUMMARY: The only symptoms and signs were fever and weak femoral pulses. The echocardiography confirmed a localised isthmic's coarctation. In order to complete the evaluation, a CT scan was performed. This proved crucial in terms of the diagnosis and decision to perform emergency surgery. The diagnosis was confirmed surgically. An aortic rupture was contained by the parietal pleura. Bacteriological analysis of surgical specimens revealed bacterial DNA that tested positive for Streptococcus pneumoniae. The post-operative course was free from any cardiovascular or neurological complications after six weeks of antibiotic therapy. DISCUSSION: Surgical repair of coarctation of the aorta is frequently performed in children. However, complicated forms are less common with a potentially fatal outcome. Indeed, there are no recommendations concerning the management and surgical timing of mycotic pseudoaneurysm. These rare complications should be kept in mind. Although short- and medium-term follow-up of these children is good, caution should be exercised with long-term follow-up because of complications in childhood and adulthood.

Tetralogy of Fallot with critical biventricular dysfunction: is surgical correction achievable?

We describe the case of a 32-month-old patient from a developing country with tetralogy of Fallot associated with a severe biventricular dysfunction. This association is rare but makes the surgical strategy complex and potentially contraindicated. An acute severe hypoxic episode led us to perform palliative rescue intervention involving the placement of an undersized systemic-to-pulmonary shunt. This surgery was well tolerated and allowed a fast and impressive recovery of the ventricular function, making complete repair possible.

Cardiac strangulation: An atypical complication from epicardial pacemaker leads in a newborn.

We report a rare and serious complication of cardiac strangulation arising from the implantation of epicardial pacing leads in a newborn. Patient's follow-up 9-month postsurgery revealed compression under the pulmonary valve annulus by a pacemaker lead, causing progressive stenosis of the right ventricular outflow tract. The epicardial leads were replaced to relieve compression, and stenosis of the right ventricular outflow tract was rectified. Pacemaker implantation in newborns is not without challenges; epicardial leads should be carefully positioned to avoid any compression of cardiac structures.

Isolated severe leftward displacement of the septum primum: anatomic and 3D echocardiographic findings and surgical repair.

OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage. METHODS: Three patients presenting with situs solitus and extreme leftward displacement of the septum primum were included. All of the pulmonary veins drained anomalously into the anatomical right atrium, and the distance between the mitral valve and the abnormal septum primum was greatly reduced, compromising the size of the left atrial chamber, but with normal left ventricle diameters. Preoperative 3D echocardiographic findings are reported. We achieved a biventricular surgical repair in all cases. The atrial septation was accomplished using an autologous pericardial patch after removing the abnormal septal membrane. RESULTS: The postoperative course was free from any cardiovascular complications. Echocardiographic scans showed a harmonious reconstruction without pulmonary venous obstructions or stenosis. CONCLUSIONS: This article reports the severe leftward displacement of the septum primum presented as an isolated cardiac malformation; 3D transthoracic echocardiography allowed an accurate diagnosis of this malformation and helped in choosing the best surgical strategy.

Assessment of Ventricular Septal Defect Size and Morphology by Three-Dimensional Transthoracic Echocardiography.

BACKGROUND: Morphologic description of ventricular septal defect (VSD) is mandatory before performing the newly developed transcatheter closure procedure. Inaccurate estimation of defect size has been reported using conventional two-dimensional (2D) transthoracic echocardiography (TTE). The aim of this study was to assess VSD morphology and size using three-dimensional (3D) TTE compared with 2D TTE and surgery. METHODS: Forty-eight children aged 21.4 ± 29.3 months with isolated muscular (n = 11 [22.9%]) and membranous (n = 37 [77.1%]) VSDs were prospectively included. Three-dimensional images were acquired using full-volume single-beat mode. Minimal diameter, maximal diameter, and systolic and diastolic VSD areas were measured from 3D data sets using multiplanar reconstruction mode (QLAB 9). Maximal-to-minimal VSD diameter ratio was used to assess VSD geometry. Linear regression analysis and the Bland-Altman method were used to compare 3D measurements with 2D and surgical measurements in a subgroup of 15 patients who underwent surgical VSD closure. RESULTS: VSD 3D diameters and areas were measured in all patients (100%; 95% CI, 92.6%-100%). Maximal diameter was lower on 2D TTE compared with 3D TTE (7.3 vs 11.3 mm, P < .0001). Mean bias was 4 mm, with 95% of values ranging from -1.76 to 9.75 mm. Correlation between 3D maximal diameter and surgical diameter was strong (r(2) = 0.97, P < .0001), while correlation between maximal 2D diameter and surgical diameter was moderate (r(2) = 0.63, P < .0001). VSDs had an oval shape when assessed by 3D TTE. Maximal-to-minimal diameter ratio assessed by 3D TTE was significantly higher in muscular VSDs compared with membranous VSDs (3.20 ± 1.51 vs 2.13 ± 1.28, respectively, P = .01). VSD area variation throughout the cardiac cycle was 32% and was higher in muscular compared with membranous VSDs (49% vs 26%, P = .0001). CONCLUSIONS: Three-dimensional TTE allows better VSD morphologic and maximal diameter assessment compared with 2D TTE. VSD shape and its changes during the cardiac cycle can be visually and quantitatively displayed. Three-dimensional echocardiography may thus be particularly useful before and during percutaneous VSD closure.

3D transthoracic echocardiography to assess pulmonary valve morphology and annulus size in patients with Tetralogy of Fallot.

BACKGROUND: Accurate evaluation of the pulmonary valve (PV) is crucial before surgical repair of Tetralogy of Fallot (TOF). AIMS: To assess PV and pulmonary annulus (PA) morphology using three-dimensional (3D) transthoracic echocardiography (TTE) in infants referred for surgical repair of TOF. Also, to compare PA measurements obtained by 3D TTE with those from other imaging modalities, including two-dimensional (2D) TTE and computed tomography (CT), with reference to surgical measurements. METHODS: 3D zoom mode was used to assess PV morphology. 2D TTE and CT PA diameters were compared to both vertical and horizontal diameters obtained from 3D datasets. Surgical PA diameters were measured using Hegar's dilators. RESULTS: A total of 29 patients with TOF (median [range] age 6 [3-24] months) were included and all successfully underwent 2D and 3D TTE; 22 also underwent CT. The number of pulmonary leaflets could be visualized in 24 patients (82.8%), with complete concordance with surgical findings. Vertical diameter was significantly longer than horizontal diameter (P<0.001)-underlying PA eccentricity-and was more important in bicuspid than tricuspid valves. Correlations between 2D and 3D TTE diameters were good. Surgical diameter was better correlated with 2D and 3D diameters than with CT diameter. 3D minimum, 2D and CT diameters were significantly lower than surgical diameters, but 3D mean and maximum diameters were not. CONCLUSION: 3D TTE is accurate to assess PV morphology and PA size in patients with TOF. 2D TTE and CT underestimate PA diameter with reference to surgical diameter, however 3D mean and maximum diameters did not differ significantly.

Left circumflex artery aneurysm: the end-to-end reconstruction.

This report describes a surgical myocardial revascularization procedure for a huge, atherosclerotic left circumflex coronary artery aneurysm. The technique proposed in this paper is based on the isolation of the aneurysm followed by the aneurysmectomy and a complete reconstruction of the circumflex artery by an end-to-end anastomosis.

Arterial Switch Operation in Single Coronary Ostium With Intramural Course: Subclavian Artery Patch Angioplasty.

This report describes a neonatal arterial switch operation in a transposition of the great arteries with single ostium and intramural course coronary pattern. The technique proposed is based on the creation of two coronary neoostia by incising the left coronary intramural course and splitting the common button followed by a patch enlargement of left coronary button using the left subclavian artery.

Late complication after repair of aortic coarctation.

BACKGROUND: Coarctation of the aorta is a congenital malformation that has long been considered completely correctable with appropriate surgery in childhood. However, with the aging of these patients, many late complications have been reported, and this notion must be reevaluated. METHODS: We retrospectively reviewed all patients who underwent reoperation between 1992 and 2012 in our adult cardiac surgery department following surgical correction of coarctation in childhood; 18 patients over 15-years old were included in the study. RESULTS: The median time from coarctation repair to reoperation was 25 years. Patients were reoperated on for several late complications: aortic valve disease secondary to bicuspid aortic valve, ascending aortic aneurysm, recoarctation, aortic arch hypoplasia, pseudoaneurysm, associated recoarctation and pseudoaneurysm, subvalvular aortic obstruction, and descending thoracic aortic aneurysm. One patient died due to an intraoperative complication. In the other cases, the surgical results were satisfactory at the 6-month follow-up. According to literature data, age at coarctation repair and surgical technique appear to be essential factors in late complications: older age and surgical repair with prosthesis interposition are associated with a higher rate of reintervention. CONCLUSION: Patients who have undergone repair of aortic coarctation frequently remain asymptomatic for a long time. Late complications can be appropriately treated when diagnosed early. Consequently, all coarctation patients need careful lifelong follow-up, especially those with congenital aortic valve disease or surgery in childhood with interposition of prosthetic material.

Giant-cell aortitis: an unusual case of Bentall operation.

Noninfectious ascending aortitis is a very rare cause of ascending aortic aneurysm. We report a case of the truly fortuitous finding of this rare condition in a 67-year-old man operated on for an ascending aortic aneurysm associated with dystrophic aortic valve regurgitation. Intraoperative inspection revealed dissection of the aorta just above the left main coronary artery. A modified Bentall operation was performed. The pathological diagnosis was giant cell arteritis.

Right origin of the circumflex artery and posterior aortic annulus enlargement.

We present the case of a patient who required posterior enlargement of the aortic annulus during aortic valve replacement in the presence of abnormal origin of the circumflex artery from the right coronary artery, with a retroaortic course.

Anomalous origin of the left main coronary artery. Surgical management.

A 53-year-old man, with no medical history, presented with acute coronary syndrome. Coronary angiography revealed a common right ostium giving rise to both the right coronary artery and the left main coronary artery. The left interior mammary artery was used as a single bypass graft to the left anterior descending coronary artery. The patient was asymptomatic on follow-up.