Section I Tetralogy of Fallot with Pulmonary Stenosis Definition Tetralogy of Fallot (TF) is a congenital cardiac malformation characterized by underdevelopment of the right ventricular (RV) infundibulum, with anterior and leftward displacement of the infundibular (conal, outlet) septum and its parietal extension. Displacement (malalignment) of the infundibular septum is associated with RV outflow (pulmonary) stenosis (in extreme forms, atresia) and a large ventricular septal defect (VSD).…

Definition Patent ductus arteriosus (PDA) is abnormal persistence of a patent lumen in the fetal ductus arteriosus, which usually connects the upper descending thoracic aorta with the proximal portion of the left pulmonary artery (LPA). When the aortic arch is right-sided, the ductus usually connects to the proximal right pulmonary artery. The ductus may at times connect to the adjacent subclavian or brachiocephalic artery rather than…

Section I Unruptured and Ruptured Sinus of Valsalva Aneurysms Definition Congenital sinus of Valsalva aneurysms are thin-walled saccular or tubular outpouchings usually located in the right sinus or adjacent half of the noncoronary sinus. They generally have an intracardiac course but may protrude into the pericardial space. They may rupture into the right (or rarely the left) heart chambers to form an aortocardiac fistula, or into…

Section I Primary Ventricular Septal Defect Definition Ventricular septal defect (VSD) is a hole or multiple holes in the interventricular septum. This chapter discusses VSDs that occur as the primary lesion, recognizing that hearts with primary VSDs may have minor coexisting morphologic abnormalities. VSD may be part of another major cardiovascular anomaly, such as tetralogy of Fallot (see Chapter 38 ), complete atrioventricular (AV) septal defect…

Definition Atrioventricular (AV) septal defects are characterized by a deficiency or absence of septal tissue immediately above and below the normal level of the AV valves, including the region normally occupied by the AV septum, in hearts with two ventricles. The AV valves are abnormal to a varying degree. These defects have also been called AV canal defects, AV defects, endocardial cushion defects, ostium primum atrial…

Definition Unroofed coronary sinus syndrome is a spectrum of cardiac anomalies in which part or all of the common wall between the coronary sinus and left atrium is absent. Hearts with atrial isomerism and a left-sided superior vena cava (LSVC) entering a left-sided atrium are included in this chapter, despite the controversy concerning proper classification of such anomalies (see “ Atrial Isomerism ” under Morphology later…

Definition Classic (or typical) cor triatriatum, or cor triatriatum sinister, is a rare congenital cardiac anomaly in which the pulmonary veins typically enter a “proximal” left atrial chamber separated from the “distal” left atrial chamber by a partition in which there are one or more restrictive ostia. 1 In the Congenital Heart Surgery Nomenclature and Database Project, cor triatriatum is classified as a pulmonary venous anomaly,…

Definition Total (totally) anomalous pulmonary venous connection (TAPVC) is a cardiac malformation in which there is no direct connection between any pulmonary vein and the left atrium; rather, all the pulmonary veins connect to the right atrium or one of its tributaries. Although not part of the malformation, a patent foramen ovale or atrial septal defect is present in essentially all persons with TAPVC and is…

Definition An atrial septal defect (ASD) is a hole of variable size in the atrial septum. A patent foramen ovale that is functionally closed by overlapping of limbic tissue superiorly and the valve of the fossa ovalis inferiorly (in response to the normal left-to-right atrial pressure gradient) is excluded. ASDs generally permit left-to-right shunting at the atrial level. Partial anomalous pulmonary venous connection (PAPVC) is a…

Definition Congenital heart disease in the adult is presence of unrepaired or repaired congenital heart disease in patients aged 21 years or older. A more practical working definition is not straightforward. Because physical and emotional maturity is variable, the distinction between an adult and non-adult is unclear. The designation “adult” implies provision of specific methods of caregiving best delivered in an adult care environment. The patient…

Definition Acquired disease of the systemic veins entails obstruction—partial or complete—of the major veins of the thorax. Veins of surgical importance are the superior and inferior venae cavae (SVC and IVC). Left and right brachiocephalic veins, including the jugular-subclavian vein confluence, are major tributaries of the SVC and may be considered collectively with conditions of the SVC. Congenital anomalies of the venae cavae and axillary vein…

In this chapter, five diseases involving the pulmonary arteries that are amenable to surgical treatment are discussed: acute massive pulmonary embolism, chronic pulmonary thromboembolic disease, pulmonary artery aneurysm, pulmonary artery dissection, and pulmonary artery tumors. Section I Acute Massive Pulmonary Embolism Definition Acute massive pulmonary embolism is sudden entrapment in pulmonary arteries of dislodged thrombus, usually from deep veins of the legs, pelvis, or arms. It…

Definition This chapter considers true aortic aneurysm, false aortic aneurysm, chronic aortic dissection, penetrating arteriosclerotic ulcer, intramural hematoma, and diffuse arteriosclerotic disease. Methods for protecting the brain during operations on the aortic arch are also discussed. True aortic aneurysm is a permanent localized dilatation of the aorta, of a diameter 50% or greater than normal, contained by walls that although attenuated, have all layers of the…

Definition Acute aortic dissection is an event of sudden onset in which blood leaves the normal aortic lumen through a usually discrete point of exit (intimal tear) and rapidly dissects inner from outer layers of the media to produce a false lumen. However, dissection of the media can occur in the absence of an intimal tear. Patients are considered to have an acute dissection when the…

Definition Acute traumatic aortic disruption is rupture of all or part of the aortic wall, usually occurring as a result of blunt trauma. A theoretical sequence of injury involves initial rupture of the intimal and medial layers. After a period of unpredictable duration, rupture of the adventitial layer occurs. Patients are considered to have acute disruption when it occurs within 14 days of injury. Chronic traumatic…

Section I Chronic Constrictive Pericarditis Definition Chronic constrictive pericarditis is a chronic inflammatory process that involves both fibrous and serous layers of the pericardium, leading to pericardial thickening and compression (constriction) of the ventricles. The resultant impairment in diastolic filling reduces cardiac function. Historical Note It is said that Galen in ad 160 described cicatricial thickening of the pericardium in an animal and surmised that the…

Definition Mechanical circulatory support (MCS) is a means of imparting energy for forward flow of blood in the body by manmade devices. Its intent is to remove some or all work of cardiac output from either or both left and right ventricles. Mechanical circulatory support devices (MCSDs) can be used to provide temporary ventricular assistance after cardiac surgery, with the assumption that ventricular function will recover…

Section I Cardiac Transplantation in Adults without Congenital Heart Disease Definition Cardiac transplantation is a therapeutic procedure whereby the heart of a suitable donor is implanted into a recipient. Historical Note Carrel and Guthrie first reported successful heterotopic cardiac transplantation in dogs in 1905. Little more was done until in 1933 Mann and colleagues at Mayo Clinic reported successful transplantation of the heart into the neck…

Cardiomyopathy is a cardiac muscle disease process that leads to clinical myocardial dysfunction. The disease process results in morphologic changes in the heart that are typically classified as (1) dilated cardiomyopathy, (2) ischemic cardiomyopathy, (3) hypertrophic cardiomyopathy, (4) restrictive cardiomyopathy, and (5) arrhythmogenic right ventricular (RV) dysplasia. Clinical manifestations, predictors of survival and functional outcome, and therapeutic objectives focus on the clinical and pathophysiologic condition termed…

Definition Hypertrophic cardiomyopathy (HCM) is a genetic myocardial disorder characterized by left and/or right ventricular hypertrophy that is usually, but not always, asymmetric and is associated with microscopic evidence of myocardial fiber disarray and fibrosis. Degree of hypertrophy at any given site can vary substantially and influences clinical manifestations of the disease. Ventricular septal hypertrophy is the most common type of asymmetric hypertrophy, with midventricular, apical,…