Grown-up Congenital Heart Disease (GUCH): Difference between revisions

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 The reported operative mortality of the Bentall procedure is 1.5% for elective and 11.7% for emergency operations. Five- and 10-year survival rates of 84 and 75%, respectively, have been reported. This relatively limited prognosis is due to late sequelae associated with MFS; 75% require reoperations, 10% mitral valve surgery, 1% develop endocarditis and 1% a CVA. Marfan syndrome has also been associated with a considerably higher risk of re-dissection and recurrent aneurysm than other aetiologies of aortic disease. Long-term results of valvesparing aortic root replacement in Marfan syndrome are still unknown.
+== Ebsteins anomaly ==
+=== Case report ===
+=== Introduction ===
+Ebsteins anomaly, named after Wilhelm Ebstein (1836 – 1912) (figure 20) is a congenital heart defect of the morphological tricuspid valve. The prevalence of Ebstein's anomaly is about 1 in 50.000 – 200.000 with a similar incidence in both males and females.
+As its name clearly indicates, the tricuspid valve consists of three leaflets; anterosuperior, septal and inferior. The Ebstein’s anomaly consists of a variety of anatomical and functional abnormalities of the tricuspid valve. Typical features are:
+* displacement of the septal and inferior leaflet downwards from the atrioventricular junction and toward the body of the right ventricle or the apex
+* the anterior leaflet is not displaced, enlarged (sail-like) and can show fenestrations
+* the tricuspid valve inlet is displaced towards the right ventricular outflow tract and often stenotic
+* atrialisation of the right ventricle because of a downward extension of the tricuspid valve, leaving a small right ventricle or only a right ventricular outflow tract
+=== Pathophysiology ===
+ The Ebstein’s anomaly can be isolated or associated with other cardiac defects like ASD or patent foramen ovale, pulmonary outflow tract obstruction, VSD, coarctation of the aorta, ccTGA, one or more accessory conduction pathways or patent ductus arteriosus.
+The primary hemodynamic consequence of Ebstein’s anomaly is tricuspid regurgitation (TR), which varies in severity. Severe TR causes a volume overload and right-sided cardiac chamber dilation and dysfunction, leading to a decrease in cardiac output. In some cases even hepatic congestion and failure can arise.
+Ebstein's anomaly can be classified as mild, moderate, or severe based upon the extent of apical displacement of the valve leaflets with resultant tricuspid regurgitation, and the degree of right ventricular dysfunction.
+Symptoms such as cyanosis and heart failure from severe TR may appear soon after birth, because of high pulmonary vascular resistance. However, symptoms often improve as pulmonary vascular resistance decreases. At a later age, symptoms such as exertional dyspnea, fatigue, cyanosis, and palpitations may recur; these symptoms may be insidious in onset. Palpitations due to atrial tachyarrhythmia are present in 20 to 30 percent of cases. Some of these arrhythmias may be due to Wolff-Parkinson-White syndrome, since up to 20 percent of patients have one or more accessory pathways; the majority of these pathways are located around the orifice of the malformed tricuspid valve. Patients with Ebstein's anomaly who have an interatrial communication are at risk for paradoxical embolization, brain abscesses, and sudden death.
+=== Treatment ===
+Indications for surgical tricuspid valve repair or replacement are:
+* decrease in exercise capacity
+* progressive cyanosis
+* progressive right ventricular dilatation or dysfunction
+* occurrence of paradoxal embolism
+* supraventricular arrhythmias despite pharmacological or ablation therapy
+* progressive cardiomegaly on chest radiograph
+There are two main surgical techniques used in tricuspid valve repair. The Danielson repair consists of horizontally plicating the atrialized portion of the RV while multiple commissuroplasty stitches are placed. The Carpentier technique consists of detaching the large sail-like anterior leaflet from the valve annulus and translocating it in a clockwise division in order to create what essentially becomes a single leaflet valve. The atrialized portion of the RV is plicated but in a vertical not a horizontal fashion. If repair is not possible and the patient has reached adult size, tricuspid valve replacement becomes necessary. A biologic prosthesis, such as a porcine valve, is usually chosen because of the high incidence of thromboembolism with a mechanical prosthesis placed in the right heart.
+=== Outcome ===
+The prognosis varies with the severity of the disease. The 1 and 10 year survival rates for all liveborn patients have been estimated 67 percent and 59 percent respectively. The major causes of death are heart failure, perioperative, and sudden death.
+However, survival is probably increasing as advances in diagnostic and surgical techniques and postoperative care have led to improvements in surgical outcome.