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Adult Congenital Heart Disease - Part 7

Lưu ý nhẹ cardiomegaly, đánh dấu nong phải tâm nhĩ và nong đánh dấu của các động mạch phổi trung ương. Điển hình xuất hiện của một bệnh nhân người lớn trẻ tuổi với phì đại tâm thất phải bồi thường và huyết áp tâm thu tâm thất phải duy trì chức năng. | 162 Chapter 20 Fig. 20.1 Chest radiograph from a patient with Eisenmenger syndrome and a large VSD. Note mild cardiomegaly marked right atrial dilatation and marked dilatation of central pulmonary arteries. Typical appearance of a young adult patient with compensated right ventricular hypertrophy and maintained right ventricular systolic function. Marked peripheral pulmonary artery prooning not present not a feaure of Eisenmenger physiology in contrast to patients with primary acquired pulmonary arterial hypertension . Course in adulthood Many individuals with Eisenmenger syndrome survive into adulthood with 80 survival at 10 years 77 survival at 15 years and 42 at 25 years after diagnosis. Variables associated with poor prognosis include syncope elevated right atrial pressure and severe resting hypoxemia 80 transcutaneous oxygen saturation . The attrition is progressive with causes of death listed below. Fig. 20.2 12-lead resting electrocardiogram EKG from the same patient with Eisenmenger syndrome and a large VSD. Note extreme right axis deviation with right ventricular hypertrophy and first-degree heart block. T wave inversion across the left and precordial leads suggests possible ischemia. Eisenmenger Syndrome 163 164 Chapter 20 Sudden death 30 Congestive heart failure 25 Hemoptysis 15 Other 30 with a partial list including - pregnancy - perioperative following non-cardiac surgery - infective endocarditis - brain abscess - non-cardiac causes. While individuals with Eisenmenger syndrome may remain relatively stable for long periods of time it is essential to appreciate that their hemodynamic state is very delicately balanced. This balance is easily upset often with disastrous results. Examination Central cyanosis with digital clubbing. Patients with a PDA may have normal-appearing nail beds on the right hand and cyanosis and clubbing of the nail beds of both feet and the left hand. Venous blood shunts through the PDA and enters the aorta distal to the right .