Left Ventricular Outflow Tract Abnormalities Research Articles

Repair for Congenital Mitral Valve Stenosis.

We report the techniques and long-term outcome of mitral valve (MV) repair to correct congenital mitral stenosis in children. Between 1986 and 2014, 137 children (mean age 4.1 ± 5.0, range 1 month-16.8 years) underwent repair of congenital mitral stenosis (CMS). In 48 patients, CMS is involved in Shone's anomaly. The typical congenital MS (type I) was seen in 56 patients. Hypoplastic MV (type II, n = 15) was associated with severe left ventricular outflow tract abnormalities and hypoplastic left ventricular cavity and muscle mass. Supravalvar ring (type III, n = 48) ranged from a thin membrane to a thick discrete fibrous ridge. Parachute MV (type IV, n = 10) have 2 leaflets and barely distinguishable commissures, but all chordae merged either into 1 major papillary muscle or asymmetric papillary muscles-1 dominant and the other minuscule. Hammock valve (type IV, n = 8) appeared dysplastic with shortened chordae directly inserted into the posterior left ventricular muscle mass. MV repair was performed using commissurotomy, chordal division, papillary muscle splitting and fenestration, and mitral ring resection, each applied according to the presenting morphology. During the 28-year follow-up period, 23 patients underwent repeat MV repair and 3 underwent MV replacement after failed attempts at repeat repair. At 1 and 15 years postoperatively, freedom from reoperation was 89.3 ± 5.1% and 52.8 ± 11.8%, and cumulative survival rates were 92.3 ± 4.3% and 70.3 ± 8.9, respectively. Mortality unrelated to repair accounted for 9 (20%) deaths. Long-term functional outcome of MV repair in children with CMS is satisfactory. Repeat repair or replacement may be deemed necessary during the course of follow-up.

Circulating mRNA in Maternal Plasma at the Second Trimester of Pregnancy: A Possible Screening Tool for Cardiac Conotruncal and Left Ventricular Outflow Tract Abnormalities.

Maternal plasma is a source of circulating placental nucleic acids. This study was designed to detect aberrantly expressed placental mRNA genes circulating in the maternal plasma of pregnancies affected with fetal conotruncal anomalies (CNTRA) and left-ventricular outflow tract (LVOT) obstruction in the second trimester of pregnancy. This was a retrospective monocentric study conducted from 1 Jan 2016 to 31 Dec 2016. NanoString technology was used to identify aberrantly expressed genes, comparing 36 women carrying a fetus with CNTRA or LVOT obstruction to 42 controls at 19-24 weeks of gestation. The genes with differential expression were subsequently tested using real-time polymerase chain reaction. Linear discriminant analysis was used to combine all the mRNA species with discriminant ability for CNTRA and LVOT obstruction. A multivariable receiver operating characteristic (ROC) curve having the estimated discriminant score as an explanatory variable was generated for the two affected groups versus controls. Three genes with differential expression, namely MAPK1, IQGAP1 and Visfatin were found. The ROC curves yielded detection rates of 60 and 62.5% at a false-positive rate of 5% for CNTRA and LVOT, respectively. These data suggested that molecular screening of CNTRA and LVOT obstruction in the second trimester is feasible. Prospective studies are needed to test the discriminant ability of these genes and to calculate the predictive positive value in the general population.

Notch-dependent EMT is attenuated in patients with aortic aneurysm and bicuspid aortic valve

Bicuspid aortic valve is the most common congenital heart malformation and the reasons for the aortopathies associated with bicuspid aortic valve remain unclear. NOTCH1 mutations are associated with bicuspid aortic valve and have been found in individuals with various left ventricular outflow tract abnormalities. Notch is a key signaling during cardiac valve formation that promotes the endothelial-to-mesenchymal transition. We address the role of Notch signaling in human aortic endothelial cells from patients with bicuspid aortic valve and aortic aneurysm. Aortic endothelial cells were isolated from tissue fragments of bicuspid aortic valve-associated thoracic aortic aneurysm patients and from healthy donors. Endothelial-to-mesenchymal transition was induced by activation of Notch signaling. Effectiveness of the transition was estimated by loss of endothelial and gain of mesenchymal markers by immunocytochemistry and qPCR. We show that aortic endothelial cells from the patients with aortic aneurysm and bicuspid aortic valve have down regulated Notch signaling and fail to activate Notch-dependent endothelial-to-mesenchymal transition in response to its stimulation by different Notch ligands. Our findings support the idea that bicuspid aortic valve and associated aortic aneurysm is associated with dysregulation of the entire Notch signaling pathway independently on the specific gene mutation.

The Ross, Konno, and Ross-Konno operations for congenital left ventricular outflow tract abnormalities.

Operations for left ventricular outflow tract abnormalities are centred on hemodynamic conditions that relate to subvalvar stenosis, valvar stenosis/regurgitation, aortic annular hypoplasia, and supravalvar aortic stenosis. Operative interventions over the years have evolved because the intervening outcomes proved to be unsatisfactory. The resection for subvalvar aortic stenosis has progressed from a fibrous "membrane" resection to a more extensive fibromuscular resection. Operative solutions for valvar aortic stenosis and regurgitation have resulted in operative interventions that depend on simple commissurotomy, leaflet extensions, prosthetic mechanical valve replacement, biologic valve replacement, including the pulmonary autograft, and operations to treat aortic annular stenosis. Although there are enthusiastic proponents for all of these strategies, the fact remains that none have proven to be curative; patients can expect to undergo further procedures during their lifetimes. The short- and mid-term solutions to these left ventricular outflow tract abnormalities have improved based on operations that have been attended by increasing operative complexity. The purpose of this review is to chronicle the operative steps of the Ross operation, the Konno-Rastan operation, the modified Konno operation, the Ross-Konno operation, and the modified Ross-Konno operation.

Left ventricular non‐compaction: Prevalence in congenital heart disease

IntroductionLeft ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease. MethodsFrom our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects. ResultsFrom a total of 202 patients with LVNC, 24 patients (12%; mean age 32±11years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni- or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni- or bicuspid aortic valves (7/963, 1%). ConclusionIn adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.

Arterial switch in hearts with left ventricular outflow and pulmonary valve abnormalities

Background. Pulmonary valve and left ventricular outflow tract abnormalities (LVOT) may not be absolute contraindications to arterial switch operation (ASO). Methods. In this study we analyze long-term outcome for 26 such transposition patients (6.3% of our ASO cohort). Median age and weight were 69 days (7 to 3,631 days) and 4.5 kg (2.6 to 34 kg). Pulmonary valve abnormalities included bicuspid valve (n = 4) and dysplastic valve (n = 5). The LVOT abnormalities (n = 17) included accessory atrioventricular valve/endocardial cushion tissue, fibromuscular ring, anomalous muscle bands, and septal malalignment. Patients with dynamic LVOT obstruction were excluded. The median preoperative left ventricular to pulmonary artery peak systolic pressure gradient was 30 mm (0 to 93 mm), or 50 mm (16 to 93 mm) if patients with isolated valve abnormalities are excluded. The ASO was performed according to our standard technique with or without LVOT resection or pulmonary valvotomy as required. Results. There were two perioperative deaths (7.7%; 95% confidence interval, 0.9% to 25%), and no late deaths during 1,934 patient-months of follow-up time. Actuarial freedom from reoperation for neoaortic valve or LVOT problems is 87% (± 7) at 130 months, representing two reoperations. One was performed for neoaortic insufficiency plus LVOT obstruction, and the other for isolated LVOT obstruction. One patient currently has significant neoaortic insufficiency, and median gradient at last follow-up is 0 mm Hg (range, 0 to 35 mm Hg). Conclusions. The ASO can be performed in selected patients with transposition of the great arteries and with LVOT abnormalities with early and late survival and functional status similar to that of matched patients with normal pulmonary valves and LVOT ( p > 0.05), but with a greater hazard for reoperation ( p < 0.05). Selection for ASO should be based on anatomic criteria rather than left ventricular to pulmonary artery gradient alone, to avoid assigning these patients with transposition of the great arteries to treatment strategies less satisfactory than ASO.Transposition, arterial switch

Cardiac index quantification by Doppler ultrasound in patients without left ventricular outflow tract abnormalities

We attempted to ascertain whether cardiac index can be directly estimated from Doppler mean velocity. Although diverse Doppler echocardiographic methods have been described for cardiac output quantification, they are not widely used in clinical practice. Cross-sectional area measurement has been identified as the main source of error in flow volume quantification. A three-phase study by Doppler echocardiography was conducted in 306 patients. In phase I, the normal mean velocity ratio of the left and right ventricular outflow tracts was established in 170 normal subjects. In phase II, cardiac index, calculated as the product of aortic annular area index by mean velocity (conventional method), and mean velocity determined in the left ventricular outflow tract and ascending aorta by pulsed and continuous wave Doppler, respectively, were correlated with thermodilution cardiac index in 66 patients. In phase III, the accuracy of the regression equations obtained was prospectively assessed in an additional 70 patients. The normal left/right ventricular outflow tract mean velocity ratio by pulsed wave Doppler was 1.1 +/- 0.1. Cardiac index (CI) calculated by the conventional method and thermodilution (TD) showed acceptable correlation (r = 0.90, CITD = 1.20 CIPWD + 357; r = 0.86, CITD = 0.90 CICWD + 262) for pulsed (PWD) and continuous wave (CWD) Doppler, respectively, but with systematic underestimation (-28 +/- 13%, p < 0.01) by pulsed wave Doppler. Mean velocity (MV) showed excellent correlation with the thermodilution cardiac index (r = 0.97, CITD = 172 MVPWD - 172; r = 0.93, CITD = 129 MVCWD - 255). When these regression equations were prospectively applied, better agreement with the thermodilution cardiac index was obtained by pulsed wave Doppler directly from mean velocity (SD 240 ml/min per m2) than when aortic annular area was considered in the calculation (SD 428 ml/min per m2). Similar results were obtained by continuous wave Doppler (SD 433 vs. 599 ml/min per m2) but with less accuracy. Left ventricular outflow tract mean velocity determined by pulsed wave Doppler permits easy, accurate cardiac index quantification in the absence of left ventricular outflow abnormalities. The simplicity of this method enhances its clinical applicability in noninvasive monitoring of cardiac index.

Improved cineangiographic evaluation of hypertrophic cardiomyopathy by caudocranial left anterior oblique view

Seven patients with hypertrophic cardiomyopathy and one with discrete subaortic stenosis were studied with axial left ventriculography (caudocranial left anterior oblique view). In addition to the angiographic findings described on conventional views, the mitral valve, ventricular septum, and posterior left ventricular wall were better profiled than with conventional views and thus better evaluated. Systolic anterior motion of the anterior leaflet of the mitral valve was readily identifiable, which is usually not the case in nonangled views. From our experience, we strongly recommend that caudocranial left ventriculography be the procedure of choice in patients suspected of left ventricular outflow tract abnormalities and that biventricular angiography be abandoned for diagnostic purposes.

Clinical Aspects of Operable Heart Disease

This text attempts to survey the field of cardiovascular surgery, summarizing the most significant developments of the past two decades. A correlation between clinical features and operative findings is well presented. Throughout the text the pathologic physiology is stressed in each case to corroborate the findings of diagnosis, natural history, and therapy. Among the 24 chapters, 20 are devoted to specific lesions, grouping aortic valvular lesions with left ventricular outflow tract abnormalities. The remaining four chapters are concerned with methods of diagnosis, extracorporeal circulation, preoperative and postoperative care, and heart transplantation. Because of the continuous expansion of the field of cardiovascular surgery, the text may be destined for rather early obsolescence as new techniques appear. For example, the treatment for persistent truncus arteriosus is discussed as being questionably surgical, whereas during the past year the operative correction of this anomaly has been successfully accomplished in a growing number of cases.