scholarly journals Supravalvulaire Aortic Stenosis: Case Report

2020 ◽  
pp. 07-11
Author(s):  
Redha Lakehal ◽  
Soumaya Bendjaballah ◽  
Khaled Khacha ◽  
Baya Aziza ◽  
Abdelmallek Brahami
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Aortic Stenosis ◽  
Coronary Sinus ◽  
Congenital Heart ◽  
Surgical Techniques ◽  
Dacron Patch ◽  
Aortic Root Enlargement ◽  
One Year

Introduction: Exceptional congenital heart disease (1 for 26000 birth) characterized by rétrécissement of aortic light. It can be isolated or part of William syndrome. The diagnosis is based on echocardiography. The intervention consists of an aortic root enlargement with Dacron patch. Surgery was indicated if gradient aortic left ventricle Superior of 50 mm hg. This clinical case is for us an opportunity to recall of this type of congenital aortic retrécissement. Methods: We reported the observation of patient 17-year-old without history presented since one-year dyspnea on exersion, palpitation and syncopes. Physical examination: murmur systolic in aortic home without other abnormalities. Chest X ray: CTI: 0.48., ECG: RSR with HVL. Echocardiography: supravalvulaire aortic stenosis; mean gradient AO-LV: 46 mm hg, LV -aortic; LV 48/26 mm + HLV, RV: 20 mm. Exploration per-opératoire: hipoplasie of the left coronary sinus, anomaly of implantation of antero -external pillar of mitral valve, aortic bicuspidie type 1, absence of coronary anomalies. It has benefit under cardio-pulmonary bypass an enlargement of the left coronary sinus according to DOTY technique with Dacron patch and conservation of aortic valve. Results: The immediate post-operative suites were favoured with gradient aortic –left ventricle drop to 20 mm hg. Conclusion: This is very rare congenital heart disease; echocardiography remains the key of diagnosis. It must be operated early. The prognostic is enhanced by the advances in surgical techniques. The treatment consists of surgery. Keywords: Supravalvulaire aortic stenosis; Surgery; Cardiopulmonary bypass

scholarly journals P642 Coronary arteriovenous fistulas

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Ciuca ◽  
A Balducci ◽  
L Lovato ◽  
F Niro ◽  
E Angeli ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Coronary Artery ◽  
Left Ventricle ◽  
Arteriovenous Fistula ◽  
Coronary Sinus ◽  
Right Atrium ◽  
Congenital Heart ◽  
Heart Diseases ◽  
The Right

Abstract Clinical case A 53 years woman in good health and un uneventful clinical history except for a mild hypercholesteremia was evaluated for palpitations. At the clinical examination she had a systolic murmur 3/6 Levine, with no signs of heart failure. The ECG showed normal sinus rhythm with a normal heart rate (62bpm), normal atrio-ventricular and intraventricular conduction and normal repolarisation, one supraventricular premature beat. The echocardiography showed normal biventricular dimension and function, no valvular heart diseases, no septal defects, regular aortic dimensions. A giant right coronary was evidenced (Figure, panel a) with an arteriovenous fistula originating from the right coronary artery and draining through the coronary sinus into the right atrium (Figure, panel b). The CT coronary angiogram evidenced an dilated right coronary artery communicating with the coronary sinus (arteriovenous fistula) draining into the right atrium. A smaller arteriovenous fistula was evidenced between the circumflex artery (slightly dilated) and the great cardiac vein. (Figure, panel c-e) The Treadmill test didn’t evidence an induced ischemia; however the patient didn’t perform a maximal exercise (double product 20400mmHb*bpm). Moreover, during the first steps of recovery frequent supraventricular premature beats were registered with phases of bigeminies followed by a junctional rhythm phase. Thus, a Gated myocardial Perfusion SPECT was performed evidenced a mild stress induced ischemia of the inferolateral and apical left ventricle wall with normal rest perfusion and normal left ventricle volumes (125ml during exercise and 134ml at rest) with a normal ejection fraction ( > 65%). (Figure, panel f)An elective coronarography was planned. The patient is on therapy with beta-blockers and aspirin. The patient is asymptomatic for angina. Antibiotic prophylaxis was recommended for dental, gastrointestinal, or urologic procedures. Discussion: Coronary arteriovenous fistula (CAVF), first described in 1865 by Krausein (1), are a rare congenital heart disease representing less than 0.5% of all congenital heart diseases with an extremely rare prevalence 0.002% in the general population (2). Moreover, therapy of CAVF is still controversial with previous data showing a relatively high rate of myocardial infarction after surgical repair (3). The recent AHA/ACC guideline for the management of adults with Congenital heart disease recommend a review by a knowledgeable team that may include congenital or noncongenital cardiologists and surgeons to determine the role of medical therapy and/or percutaneous or surgical closure (4) Conclusion: CAVF is a very rare congenital heart defect and might be asymptomatic and evidenced by hazard in adults patients. Therapy strategy demands a multidisciplinary team evaluation and should be be individualized according to the clinic presentation, the presence or absence of myocardial ischemia or ventricular dysfunction. Abstract P642 Figure.

Ultrasound screening for congenital heart disease: A case of aortic stenosis

2016 ◽  
Vol 76 (05) ◽  
Author(s):  
F Mraihi ◽  
A Gharsa ◽  
S Schlomann ◽  
A Achour ◽  
D Chelly
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Aortic Stenosis ◽  
Congenital Heart ◽  
Ultrasound Screening

Double-chambered left ventricle with complex congenital heart disease in an infant: an extremely rare pathology

2019 ◽  
Vol 16 (3) ◽  
pp. 187-191
Author(s):  
T.V. Rogova ◽  
A.I. Kim ◽  
A.V. Sobolev ◽  
S.A. Aleksandrova ◽  
E.V. Kholmanskaya ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease

scholarly journals Pattern and Clinical Profile of Congenital Heart Disease in A Teaching Hospital

1970 ◽  
Vol 21 (1) ◽  
pp. 58-62 ◽  
Author(s):  
L Shamima Sharmin ◽  
M Azizul Haque ◽  
M Iqbal Bari ◽  
M Ayub Ali
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Hospital Stay ◽  
Congenital Heart ◽  
Growth Failure ◽  
Female Child ◽  
Feeding Problems ◽  
Medical College ◽  
One Year ◽  
A Prospective Study

Objectives: To see the (a) type of congenital heart disease, (b) Clinical presentation of the cases, (c) association with extra-cardiac anomalies and disease, (d) complications of different CHD, (e) outcome of patients during hospital stay. Methodology: it was a prospective study conducted in the department of pediatrics of Rajshahi Medical College & Hospital over a period of one year.115 children from birth to 12 years of age who had congenital heart disease confirmed by echocardiography were included. All patients were treated conservatively and observed for immediate out come during the hospital stay. Result: major types of CHD were VSD (42.6%), TOF (18.3%), ASD (14.8%), PDA (7.8%). Male outnumbers female child. Common symptoms were breathlessness (60%), fatigue (54.8%), cough (43.5%), poor weight gain (41.7%), recurrent chest infection (34.8%), fever (28.7%), feeding problems (26.1%), palpitation (21.7%) and bluish discoloration of lips and fingertips (20%). Murmur with or without thrill and cardiomegaly was the most important cardiac finding. Frequently observed complications were heart failure, pulmonary hypertension and growth failure.   doi: 10.3329/taj.v21i1.3221 TAJ 2008; 21(1): 58-62

scholarly journals Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

Catheter Ablation and Device Therapy in Congenital Heart Disease

2013 ◽  
pp. 203-214
Author(s):  
Chenni S. Sriram ◽  
Malini Madhavan ◽  
Peter A. Brady ◽  
Bryan C. Cannon ◽  
Christopher J. McLeod ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Catheter Ablation ◽  
Congenital Heart ◽  
Surgical Techniques ◽  
Cardiac Anatomy ◽  
Anatomic Variations ◽  
Device Management ◽  
Surgical History ◽  
Major Increase

Arrhythmias in adult CHD patients represent the leading cause of morbidity and hospitalization, resulting in a major increase in ablation and device-related procedures in these patients. These procedures are often complex, not only because of underlying congenital anatomic variations but also because of the effects of prior surgical repair that can modify the arrhythmic substrate leading to an increased likelihood of multiple types of arrhythmias, even in the same patient. To optimize both success and safety with these procedures, the electrophysiologist must be thoroughly familiar with the underlying cardiac anatomy, prior cardiac surgical history, and known variants of both the pathology and surgical techniques. The purpose of this chapter is to outline the salient concepts and principles pertinent to arrhythmia and device management in patients with CHD.

Adult congenital heart disease

2016 ◽  
Author(s):  
Giovanni Di Salvo ◽  
Werner Budts ◽  
Owen I. Miller
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Low Cost ◽  
Adult Congenital Heart Disease ◽  
Surgical Techniques ◽  
Adult Congenital ◽  
First Time ◽  
Established Role ◽  
Typical Scenario

Echocardiography has a well-established role in the assessment of adults with congenital heart disease. It provides accurate anatomical and functional information and is a bedside, low-cost, and easily repeatable technique. Although some lesions may be diagnosed for the first time in adulthood or may not need a surgical repair, in most cases the typical scenario is to evaluate an adult patient with a known congenital heart disease who has already undergone one (or more) previous surgical or catheter interventions, often during childhood. This chapter provides the basic anatomical concepts for the most frequent diseases, together with a brief presentation of the most used surgical techniques and the haemodynamic patterns seen after these corrections. However, the chapter is only an overview due to the variety of congenital heart disease and the complexity of its imaging.

Atrial septal defects (ASDs)

2011 ◽  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Atrial Septal Defect ◽  
Coronary Sinus ◽  
Patent Foramen Ovale ◽  
Congenital Heart ◽  
Septal Defect ◽  
Foramen Ovale ◽  
Septal Defects ◽  
Different Types

Introduction 94Ostium secundum ASD 96Ostium primum ASD 100Sinus venosus ASD 100Coronary sinus defect 102Patent foramen ovale 104Interatrial communications account for ~10% of congenital heart disease. Different types of atrial septal defect (ASD) are illustrated in Fig. 8.1.•...

Sign in / Sign up

Export Citation Format

Share Document