P3735Incidence of bicuspid aortic valve in 16,185 neonates by echocardiographic screening in a single institution: is it really congenial?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
K Waki ◽  
K Ueda ◽  
K Satoh ◽  
K Ogino ◽  
T Hayashi ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Coarctation Of The Aorta ◽  
Later Life ◽  
Population Based ◽  
True Incidence ◽  
Incidence Data ◽  
The Right

Abstract Background Bicuspid aortic valve (BAV) has been reported to be the most common congenital heart disease with an incidence of 1 to 2% in the general population; however, its incidence in neonates is still unclear because most of the reported incidence data are based on surgical or autopsy cases in adults. Purpose To elucidate the true incidence of congenital isolated BAV in neonates by echocardiographic screening in a population-based study. Methods We examined a total of 16,185 full-term neonates (male, 48.9%) born in our institution during either of the following two periods: September 1986 to February 2008 and October 2014 to September 2017. Mean gestational age (range) was 39.4 (36 to 42) weeks, and mean birth weight (range) was 3075 (2268 to 4622) g. On the second day after birth, we performed echocardiography and colour-Doppler flow mapping in all subjects. BAV was classified into two types on the basis of morphologicfindings in the parasternal short axis view of the aortic valve: BAV with raphe harbouring two cusps and commissures, showing a congenital fusion of two underdeveloped cusps, and BAV without raphe, showing a fish mouth appearance in systolic images. BAV associated with other congenital heart diseases such as coarctation of the aorta was excluded from this study. Results BAV was identified in 14 neonates (0.09%), an incidence of 0.9 in 1,000 live births. Of the 14 BAV neonates, five had BAV with raphe, whereas nine had BAV without raphe. Of the five BAV neonates with raphe, four had fusion of the right and noncoronary cusps, whereas one had that of the right and left coronary cusps. No aortic regurgitation was detected except trivial one in only one neonate, and no significant valvar stenosis was detected. Conclusion The incidence of BAV in neonates was much lower than previously reported incidence data. An acquired fusion of the cusps may develop in later life, and eventually may result in increased incidence of functional BAV.

scholarly journals Cardiac disease and pregnancy: hyper vigilance and extreme caution for optimal outcome

2020 ◽  
Vol 9 (2) ◽  
pp. 825
Author(s):  
Naresh Dhawan ◽  
Rohin Kumar ◽  
Reema Kumar Bhatt
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Cardiac Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Diseases ◽  
Fetal Outcome ◽  
Coordinated Care ◽  
Extreme Caution ◽  
Congenital Cardiac Malformation

Cardiac disease in pregnancy is a leading cause of maternal death in more so high-income countries. The armamentarium for winning this difficult battle involves shared decision-making with communication across the clinical team and the patient. There is limited clinical evidence concerning effective approaches to managing such complex care and moreover involvement of different specialists makes coordinated care challenging. Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, occurring in 1-2% of the population whereas a single ventricle is a rare congenital heart disease that accounts for less than 1% of all congenital heart diseases. We had two cases of pregnancy with bicuspid aortic valve in one case and the other with single ventricle. The involvement of multidisciplinary team involving cardiologist, cardiothoracic anaesthetist and fetal maternal medicine specialist resulted in good maternal and fetal outcome in both the cases.

scholarly journals Urinary Metabolomics Study of Patients with Bicuspid Aortic Valve Disease

Molecules ◽  
2021 ◽  
Vol 26 (14) ◽  
pp. 4220
Author(s):  
Massimo Chessa ◽  
Mario Panebianco ◽  
Sara Corbu ◽  
Milena Lussu ◽  
Angelica Dessì ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Metabolic Pathways ◽  
Congenital Heart ◽  
Roc Analysis ◽  
Operating Characteristic ◽  
Molecular Fingerprint ◽  
Metabolomics Study ◽  
Cardiovascular Anomalies ◽  
Potential Use

Bicuspid aortic valve (BAV) is the most common congenital heart defect responsible for valvular and aortic complications in affected patients. Causes and mechanisms of this pathology are still elusive and thus the lack of early detection biomarkers leads to challenges in its diagnosis and prevention of associated cardiovascular anomalies. The aim of this study was to explore the potential use of urine Nuclear Magnetic Resonance (NMR) metabolomics to evaluate a molecular fingerprint of BAV. Both multivariate and univariate statistical analyses were performed to compare the urinary metabolome of 20 patients with BAV with that of 24 matched controls. Orthogonal partial least squared discriminant analysis (OPLS-DA) showed statistically significant discrimination between cases and controls, suggesting seven metabolites (3-hydroxybutyrate, alanine, betaine, creatine, glycine, hippurate, and taurine) as potential biomarkers. Among these, glycine, hippurate and taurine individually displayed medium sensitivity and specificity by receiver operating characteristic (ROC) analysis. Pathway analysis indicated two metabolic pathways likely perturbed in BAV subjects. Possible contributions of gut microbiota activity and energy imbalance are also discussed. These results constitute encouraging preliminary findings in favor of the use of urine-based metabolomics for early diagnosis of BAV.

scholarly journals Bicuspid aortic valve morphology and aortic valvular outflow jets: an experimental analysis using an MRI-compatible pulsatile flow circulation system

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Kaoru Hattori ◽  
Natsuki Nakama ◽  
Jumpei Takada ◽  
Gohki Nishimura ◽  
Ryo Moriwaki ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Pulsatile Flow ◽  
Aortic Wall ◽  
Ascending Aorta ◽  
Circulation System ◽  
Resonance Imaging ◽  
Left Posterior ◽  
Flow Circulation ◽  
The Right

AbstractThe characteristics of aortic valvular outflow jet affect aortopathy in the bicuspid aortic valve (BAV). This study aimed to elucidate the effects of BAV morphology on the aortic valvular outflow jets. Morphotype-specific valve-devising apparatuses were developed to create aortic valve models. A magnetic resonance imaging-compatible pulsatile flow circulation system was developed to quantify the outflow jet. The eccentricity and circulation values of the peak systolic jet were compared among tricuspid aortic valve (TAV), three asymmetric BAVs, and two symmetric BAVs. The results showed mean aortic flow and leakage did not differ among the five BAVs (six samples, each). Asymmetric BAVs demonstrated the eccentric outflow jets directed to the aortic wall facing the smaller leaflets. In the asymmetric BAV with the smaller leaflet facing the right-anterior, left-posterior, and left-anterior quadrants of the aorta, the outflow jets exclusively impinged on the outer curvature of the ascending aorta, proximal arch, and the supra-valvular aortic wall, respectively. Symmetric BAVs demonstrated mildly eccentric outflow jets that did not impinge on the aortic wall. The circulation values at peak systole increased in asymmetric BAVs. The bicuspid symmetry and the position of smaller leaflet were determinant factors of the characteristics of aortic valvular outflow jet.

High prevalence of ascending aortic dilation in adults with repaired coarctation of the aorta

2021 ◽  
pp. 1-6
Author(s):  
Daniel Rinnström ◽  
Mikael Dellborg ◽  
Ulf Thilén ◽  
Peder Sörensson ◽  
Niels-Erik Nielsen ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Aortic Valve ◽  
Aortic Valve Disease ◽  
Congenital Heart ◽  
Coarctation Of The Aorta ◽  
Valve Disease ◽  
Aortic Dilation ◽  
Factors Associated ◽  
High Prevalence ◽  
Life Threatening

Abstract Background: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients. Methods and results: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]). Conclusions: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

scholarly journals TETRALOGI FALLOT DAN ATRESIA PULMONAL

2013 ◽  
Vol 4 (3) ◽  
Author(s):  
Alice I. Supit ◽  
Erling D. Kaunang
Keyword(s):  
Patent Ductus Arteriosus ◽  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Ductus Arteriosus ◽  
Ventricle Hypertrophy ◽  
The Right ◽  
Right Ventricle Hypertrophy ◽  
Patent Ductus

Abstract: Congenital heart disease is a structural defect due to the malformation of the heart, aorta, and or great blood vessels. It is the most frequent congenital malformation in newborn babies. Tetralogy of Fallot is one of the congenital heart diseases (CHD) with central cyanosis, and covers 5-10% of all CHD. We reported a boy of one year old with Tetralogy of Fallot and pulmonal atresia (ToF-PA), associated with bronchopneumonia. The diagnosis was based on anamnesis, physical examination, and other supporting examinations. The chest X-ray showed a normal sized heart (CTR 57%) with coer-en-sabot shape, and right and left parahilar infiltration, which resulted in bronchopneumonia and ToF. The electrocardiography showed a right deviation of axis and a hypertrophy of the right ventricle; the echocardiography showed a right ventricle hypertrophy, an over-riding aorta, a large malalignment of the ventricular septal defect, no visualization of pulmonar artery, and no visualization of patent ductus arteriosus (PDA). Conclusion: Based on all the tests performed, the diagnosis of this patient was Tetralogy of Fallot and pulmonal atresia (ToF-PA), associated with bronchopneumonia. The prognosis related to bronchopneumonia in this case was good due to the use of antibiotics. Keywords: tetralogy of Fallot, pulmona atresia, bronchopneumonia.  Abstrak: Penyakit jantung bawaan (PJB) ialah kelainan struktural akibat malformasi jantung, aorta dan atau pembuluh darah besar, dan merupakan kelainan kongenital tersering pada bayi baru lahir. Tetralogi Fallot merupakan salah satu PJB dengan sianosis sentral, dan mencakup 5-10% dari seluruh PJB. Kami melaporkan kasus seorang anak laki-laki berusia satu tahun dengan Tetralogi Fallot dan atresia pulmonal (ToF-PA) disertai bronkopneumonia. Diagnosis ditegakkan melalui anamnesis, pemeriksaan fisik, dan pemeriksaan penunjang. Hasil ekspertisi foto toraks AP memperlihatkan ukuran jantung normal (CTR 57%) berbentuk coer-en-sabot, dan pada paru-paru terlihat infiltrat parahilar kanan dan kiri serta corakan vaskular paru berkurang yang menunjukkan suspek bronkopneumonia dan ToF. Elektrokardiografi memperlihatkan deviasi aksis ke kanan dan hipertrofi ventrikel kanan, dan pada ekokardiografi tampak right ventricle hypertrophy, overriding aorta, VSD malalignment besar, tidak tampak visualisasi arteri pulmonal, dan tidak tampak patent ductus arteriosus (PDA) dengan hasil Tetralogi Fallot dan atresia pulmonal. Simpulan: Berdasarkan hasil pemeriksaan yang dilakukan, diagnosis pasien ini ialah Tetralogi Fallot dan atresia pulmonal (ToF-PA) disertai bronkopneumonia. Prognosis bronkopenumonia pada kasus ini baik yang dapat diatasi dengan antibiotika.Kata kunci: tetralogi Fallot, atresia pulmonal, bronkopneumonia.  

scholarly journals An infrequent association: Left ventricular non compaction with bicuspid aortic valve and coarctation of the aorta.

2020 ◽  
Vol 4 (1) ◽  
Author(s):  
Aureo Campos-Tello ◽  
Giancarlo A Valle ◽  
Felix A Revilla ◽  
Sara Rami­rez-Flores ◽  
Ãngel CuevaParra ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Coarctation Of The Aorta ◽  
Left Ventricular
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