A patient with Turner's syndrome associated with unexplained left ventricular hypertrophy, severe left ventricular systolic dysfunction, atrial septal defect and pericardial effusion/Aciklanamayan sol ventrikul hipertrofisi, ciddi sol ventrikul sistolik disfonksiyonu, atriyal septal defekt ve perikardiyal efuzyon birlikteligi olan Turner sendromlu bir hasta.A 40-year-old female with Turner's syndrome presented with a 3 months history of progressive dyspnea and peripheral edema. Physical examination revealed signs of biventricular failure. Blood pressure was 110/70 mmHg. The patient had been seen by physicians previously and there had been no signs of hypertension. Electrocardiogram showed left and right atrial dilatation, left ventricular hypertrophy left ventricular hypertrophy Cardiology Enlargement of the left ventricle often linked to the prolonged hemodynamic stress of CHF, characterized by myocardial cell hypertrophy, ↑ left ventricular wall thickness, ↓ ventricular compliance, ↑ and ST depressions in the infero-lateral derivations. Transthoracic echocardiography images revealed left ventricular hypertrophy (interventricular septum: 15 mm, posterior wall: 22 mm), severe left ventricular systolic SystolicThe phase of blood circulation in which the heart's pumping chambers (ventricles) are actively pumping blood. The ventricles are squeezing (contracting) forcefully, and the pressure against the walls of the arteries is at its highest. dysfunction (ejection fraction: 10%), dilated left atrium (50 mm), moderate pericardial effusion (Fig. 1), secundum atrial septal defect Atrial Septal Defect Definition An atrial septal defect is an abnormal opening in the wall separating the left and right upper chambers (atria) of the heart. (Fig. 2), moderate mitral and tricuspid regurgitations, pulmonary hypertension (pulmonary artery pressure: 50 mmHg), left ventricular diastolic dysfunction (mitral A wave>E wave) and dilations of the right heart chambers. A medical therapy including beta- blocker, diuretic, angiotensin converting enzyme Noun 1. angiotensin converting enzyme - proteolytic enzyme that converts angiotensin I into angiotensin II angiotensin-converting enzyme, ACE peptidase, protease, proteinase, proteolytic enzyme - any enzyme that catalyzes the splitting of proteins into inhibitor and digitalis digitalis (dĭj'ĭtăl`ĭs), any of several chemically similar drugs used primarily to increase the force and rate of heart contractions, especially in damaged heart muscle. The effects of the drug were known as early as 1500 B.C. was commenced. [FIGURES 1-2 OMITTED] There is high prevalence of congenital heart defects Congenital heart defects Congenital means conditions which are present at birth. Congenital heart disease includes a variety of defects that babies are born with. Mentioned in: Heart Failure, Heart Surgery for Congenital Defects in patients with Turner's syndrome. Aortic malformations, including bicuspid aortic valve bicuspid aortic valve Cardiology A congenital heart defect in which the aortic annulus has 2 instead of 3 semilunar valves, seen in 3% of the population; ♂:♀, 4:1; 20% of those with a BAV have other cardiovascular disease–eg, PDA or aortic , coarctation of the aorta Coarctation of the Aorta Definition A defect that develops in the fetus in which there is a narrowing of the aortic arch, the main blood artery that delivers blood from the left ventricle of the heart to the rest of the body. and aortic dilation are the most common. Hypertension, which may cause left ventricular hypertrophy is frequent in Turner's syndrome (1). However there was no hypertension in our patient. The patient had been seen by physicians previously and there had also been no hypertension as a cause of left ventricular hypertension. Although atrial septal defect is frequent in Noonan syndrome, it is extremely rare in Turner's syndrome (2). Pericardial effusion has never been reported in Turner's syndrome. References (1.) Lin AE, Lippe B, Rosenfeld RG. Further delineation of aortic dilation, dissection, and rupture in patients with Turners syndrome. Pediatrics 1998; 102: e12. (2.) Pyeritz RE. Genetics and cardiovascular disease. In: Braunwald E, Zipes DP and Libby P, editors: Heart Disease: a Textbook of Cardiovascular Medicine. 6th ed. Philadelphia: WB Saunders; 2001. p.1977-2010. Address for Correspondence: Dr. Mehmet Ozaydin, Sevket Demirel Kalp Merkezi, Kardiyoloji Anabilim Dali, Isparta, Turkey Gsm: +90 532 413 95 28 Fax: +90 246 232 45 10 E-mail: drmehmetozaydin@yahoo.com Mehmet Ozaydin, Ercan Varol ([section]), Huseyin Okutan *, Oktay Peker *, Abdullah Dogan, Ahmet Altinbas ([section]), Halil Kahraman From the Departments of Cardiology and * Cardiovascular Surgery, Suleyman Demirel University, Isparta ([section]) From the Department of Cardiology, Isparta State Hospital, Isparta, Turkey |
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