A Comparative Study of Histopathological Changes in the Ascending Aorta and the Risk Factors Related to Histopathological Conditions and Aortic Dilatation in Patients With Tetralogy of Fallot and a Functionally Univentricular Heart.

BACKGROUND: The purposes of this study were to prospectively evaluate the histologic characteristics of the aortic wall of patients undergoing univentricular type of repair and compare the same with the findings observed in patients undergoing intracardiac repair of tetralogy of Fallot (TOF). PATIENTS AND METHODS: Operatively excised full-thickness aortic wall tissue from 99 consecutive patients undergoing either intracardiac repair of TOF (group I; n=42) or univentricular repair (group II; n=57) were studied by light microscopy. Age at operation was 13 months to 28 years (mean 99.97±73.21months) for group I and 9 months to 25 years (mean 79.52±60.09) months for group II patients. RESULTS: Dilatation of the ascending aorta was present in 85.7% patients with TOF and 91.2% patients with a univentricular heart. Seventeen (17.2%) aortic specimens were histologically normal and were used as normal controls (group I, n=5; group II, n=12). A lamellar count of less than 60 was associated with a sensitivity of 97.2% and a specificity of 66.7% in patients undergoing repair of TOF and a sensitivity of 84.6% and a specificity of 80% in patients undergoing univentricular type of repairs respectively. Patients undergoing intracardiac repair of TOF and those undergoing univentricular repair exhibited 23.67 times (15.91-147.40) and 8.48 times (3.62-15.84) increased risk of aortic dilatation respectively. CONCLUSIONS: Our findings indicate the existence of significant elastic fragmentation, muscle disarray, medionecrosis and fibrosis involving the ascending aortic media in patients with a functionally univentricular heart and dilated aorta. These histopathological changes are similar to those encountered in patients with TOF and dilated aorta.

An alternative technique for rechanneling of sinus venosus atrial septal defect with partial anomalous pulmonary venous connection using autogenous right atrial appendage.

We report a new technique for closure of sinus venosus atrial septal defect with high partial anomalous pulmonary venous connection. This technique consisted of preservation of the atriocaval junction, advancement of the posterior rim of the atrial septal defect anterosuperiorly and enlargement of the superior caval vein using right atrial appendage. We found this to be a convenient technique and recommend it for correction of this anomaly.

Surgical repair of D-TGA with an aortopulmonary window and ventricular septal defects / Correção cirúrgica da transposição das grandes artérias com janela aortopulmonar e defeitos do septo ventricular

D-Transposition of great arteries with an aortopulmonary window is a rare congenital anomaly. We describe a case of D-Transposition of great arteries with an aortopulmonary window and multiple ventricular septal defects in a 5-month boy who underwent successful surgical repair.

A transposição das grandes artérias com uma janela aortopulmonar é uma anomalia congênita rara. Descrevemos um caso de transposição das grandes artérias com janela aortopulmonar e múltiplos defeitos do septo ventricular em um menino de 5 meses submetido a tratamento cirúrgico com sucesso.

Histomorphometric analysis of intrapulmonary vessels in patients undergoing bidirectional Glenn shunt and total cavopulmonary connection.

OBJECTIVES: The purposes of this study were to elucidate the histomorphometry of the intrapulmonary arteries in patients undergoing univentricular type of repairs and to identify the histomorphometric characteristics, if any, that may predispose patients to postoperative Fontan failure. PATIENTS AND METHODS: Operatively resected wedges of lung tissue from 44 patients undergoing univentricular type of repairs (aged 17 months to 34 years; mean, 83.52 ± 75.90 months) was subjected to histomorphometric analysis. RESULTS: Despite pulmonary arterioplasty, a low Nakata index was associated with 9.61 (95% confidence interval: 1.01-91.5; P = .003) times increased risk of death after the operation. A statistically significant difference in the mean indexed diameter of the intra-acinar pulmonary arteries (P = .03) was observed between patients undergoing superior and total cavopulmonary connections. Overall, there were 8 (8.2%), 4 (9.1%), 13 (29.5%), and 29 (65.9%) instances of intrapulmonary arterial intimal lesions, thrombosis, smooth muscle extension, and interstitial fibrosis, respectively. Among patients undergoing total cavopulmonary connection, only low Nakata index was significantly associated with the presence of severe intimal lesions, abnormal smooth muscle extension, intra-acinar pulmonary arterial thrombus, and smaller intra-acinar pulmonary arteries. CONCLUSIONS: A low Nakata index is significantly associated with the presence of severe intimal lesions, thrombus, abnormal smooth muscle extension, a lower mean indexed area of the intrapulmonary arteries, and poor postoperative outcome. However, none of the histomorphometrically derived parameters could conclusively predict the outcome after univentricular repair.

Relationship of internal jugular venous oxygen saturation and perfusion flow rate in children and adults during normothermic and hypothermic cardiopulmonary bypass.

INTRODUCTION: This study was designed to elucidate the trends in cerebral venous oxygen saturation in cyanotics and acyanotics undergoing normothermic and hypothermic cardiopulmonary bypass (CPB) and its relationship to perfusion flow rates. METHODS: Five hundred and forty-eight patients (253 cyanotics) undergoing first surgical correction using CPB were included in this prospective study. One hundred and seventy-two patients underwent surgical correction under normothermic CPB (34-36 degrees C) - group I; 142 patients were operated under moderately hypothermic CPB - group II; and 234 patients were operated under deep hypothermic CPB - group III. The perfusion flow rates were adjusted to maintain the internal jugular venous oxygen saturation (IJVO2) between 70-80% in both cyanotics and non-cyanotics. RESULTS: The prevalence of preoperative cerebral venous desaturation was 17.4% and 5.1% in cyanotic and acyanotic groups, respectively. All patients undergoing hypothermic CPB had IJVO 2 >75% at the recommended perfusion flow rate. During surgery, 87.2% of group I patients undergoing normothermic CPB and 88.5% of group II and III patients undergoing hypothermic CPB had IJVO 2 <75% during re-warming and required an increased perfusion flow rate to maintain IJVO2 >75%. The cyanotics demonstrated a higher incidence of cerebral desaturation in all three groups. Patients aged <4 years had almost the same prevalence of cerebral desaturation compared to the older patients. CONCLUSIONS: We conclude that patients undergoing normothermic CPB are at greater risk of cerebral desaturation. The cyanotics are at greater risk compared to acyanotics during normothermic CPB and during the re-warming phase of hypothermic CPB and require an individualised increased perfusion flow rate.

Salvage one and one-half ventricular repair and resection of Ewing's sarcoma of cardiac origin.

A 4-year-old boy with primary cardiac Ewing's sarcoma presenting with congestive cardiac failure is reported for its rarity. Its surgical importance is highlighted.

Surgical repair of D-TGA with an aortopulmonary window and ventricular septal defects.

D-Transposition of great arteries with an aortopulmonary window is a rare congenital anomaly. We describe a case of D-Transposition of great arteries with an aortopulmonary window and multiple ventricular septal defects in a 5-month boy who underwent successful surgical repair.

An unusual case of dissecting aneurysms involving both coronary sinuses of valsalva.

A 16-year-old boy with a progressive history of dyspnea and palpitations was diagnosed to have rupture of the right and left coronary sinuses on transthoracic echocardiography. The right coronary sinus had ruptured and dissected into the interventricular septum from its base up to the left ventricular apex and all around the lateral wall of the left ventricle up to the base of the papillary muscles. The left coronary sinus was also ruptured, and the rupture was contained by a false aneurysm that dissected into the roof of the left atrium from the aortomitral intervalvular fibrosa. The involvement of multiple coronary sinuses is extremely rare.

Successful aneurysmectomy of a congenital apical left ventricular aneurysm.

Congenital apical left ventricular aneurysm is a rare clinical entity that is different from congenital left ventricular diverticulum. This aneurysm usually occurs as an isolated anomaly. Its clinical presentation varies, and it is usually diagnosed by exclusion. Herein, we report the case of a 54-year-old man who experienced progressively increasing symptoms of congestive cardiac failure. Through the use of contrast echocardiography and angiocardiography, and upon histopathologic examination, he was diagnosed to have a congenital apical left ventricular aneurysm. He was successfully treated by means of left ventricular aneurysmectomy. We discuss the process of diagnosis and surgical correction of the aneurysm, and we briefly review the pertinent medical literature.

Central pulmonary artery histopathology in patients with cyanotic congenital heart diseases.

BACKGROUND: This study evaluated the pulmonary arterial (PA) wall histopathology and the risk factors related to histopathology and inadequate pulmonary artery growth in patients with cyanotic congenital heart diseases (CHDs) undergoing primary or second-stage operations after different types of systemic-to-PA shunts with or without pulmonary arterioplasty. METHODS: Operatively excised full-thickness PA tissue from 150 consecutive patients (mean age, 90.8 +/- 62.8 months; range, 6 months to 47 years) undergoing primary or second-stage procedures for different cyanotic CHDs were studied by light microscopy. The controls were 43 patients (23.8%) with normally developed central PAs and normal wall histology. RESULTS: The incidence of elastic fragmentation, increased ground substance, smooth muscle disarray, intimal thickening, and fibrosis was 61.3%, 36.6%, 34.6%, 39.3%, and 42% respectively. The risk of inadequate PA growth was 174.8, 64.7, and 45.5 times higher in patients with muscle disarray of the PA, abnormal lamellar count, and absence of palliative shunting procedures. CONCLUSIONS: Almost all PA walls in shunted patients undergoing primary/second-stage procedures for cyanotic CHD indicate significant lamellar loss and intrinsic pulmonary arteriopathy. These changes are present in infancy, are more pronounced in patients with deep cyanosis, and may account for or may coexist with a higher incidence of PA hypoplasia and inadequate PA growth encountered in these patients.

Myocardial injury in coronary artery bypass grafting: on-pump versus off-pump comparison by measuring high-sensitivity C-reactive protein, cardiac troponin I, heart-type fatty acid-binding protein, creatine kinase-MB, and myoglobin release.

OBJECTIVES: We sought to investigate the release pattern of different cardiac biomarkers (high-sensitivity C-reactive protein, cardiac troponin I, heart-type fatty acid-binding protein, creatine kinase-MB, and myoglobin) and to establish the diagnostic discrimination limits of each marker protein to evaluate perioperative myocardial injury in patients undergoing coronary artery bypass grafting with or without cardiopulmonary bypass. METHODS: Fifty patients were randomly assigned to on-pump or off-pump coronary artery bypass grafting. All cardiac biomarkers were measured in serial venous blood samples drawn before heparinization in both groups and after aortic unclamping at 1, 2, 4, 8, 24, 48, and 72 hours in the on-pump group. In the off-pump group samples were taken after the last distal anastomosis and at same time intervals as in the on-pump group. RESULTS: The total amount of heart-type fatty acid-binding protein, cardiac troponin I, and high-sensitivity C-reactive protein released was significantly higher in the on-pump group than in the off-pump group. Receiver operating characteristic curve analysis of cardiac biomarkers indicated cardiac troponin I and heart-type fatty acid-binding protein as the superior diagnostic discriminators of myocardial injury, with an optimal cutoff value of greater than 0.92 ng/mL (area under the curve, 0.95 [95% CI, 0.88-1.00]; sensitivity, 92%; specificity, 92%; likelihood ratio [+], 11.50) and greater than 6.8 ng/mL (area under the curve, 0.94 [95% CI, 0.88-1.00]; sensitivity, 88%; specificity, 88%; likelihood ratio [+], 7.33), respectively. Logistic regression analysis revealed that patients with increased cardiac troponin I levels of greater than 0.92 ng/mL and heart-type fatty acid-binding protein levels of greater than 6.8 ng/mL were at 132.25 (95% confidence interval, 17.14-1020.49) times and 53.77 (95% confidence interval, 9.76-296.12) times higher risk of myocardial injury after on-pump coronary artery bypass grafting. CONCLUSIONS: Off-pump coronary artery bypass grafting provides better myocardial protection than on-pump coronary artery bypass grafting. Cardiac troponin I and heart-type fatty acid-binding protein, but not high-sensitivity C-reactive protein, served as superior diagnostic discriminators of perioperative myocardial damage after on-pump coronary artery bypass grafting.

Histopathologic changes in ascending aorta and risk factors related to histopathologic conditions and aortic dilatation in patients with tetralogy of Fallot.

OBJECTIVE: The purposes of this study were to evaluate the histologic characteristics of the aortic wall and the risk factors related to histopathology and aortic dilatation in patients undergoing intracardiac repair of tetralogy of Fallot. METHODS: Operatively excised full-thickness aortic wall tissue from 98 consecutive patients undergoing intracardiac repair of tetralogy of Fallot aged 6 months to 47 years (mean 104.5 +/- 102.8 months; median 72 months) were studied by light microscopy. The receiver operating characteristic curve analysis was done to quantify the diagnostic accuracy of loss of lamellar counts and multiple logistic regression models. RESULTS: Twenty-five (25.5%) aortic tissue specimens were indicated as histologically normal and were used as normal controls. The incidence of elastic fragmentation, increased ground substance, medionecrosis, smooth muscle disarray, and fibrosis was 74.5%, 54%, 39.8%, 26.5%, and 57.1%, respectively. A lamellar count of less than 60 was associated with a sensitivity of 80% and a specificity of 87.67%. Area under the receiver operating characteristic curve indicated that 93.37% (standard error +/- 0.039) of the time the value of lamellar count was lower for the abnormal histopathology group than for the normal group (P < .001). The risk of aortic dilatation was 15.97 times higher in patients with histopathologically abnormal aorta. CONCLUSIONS: The majority of aortic media of the ascending aorta in cyanotic tetralogy of Fallot indicates significant loss of lamellar units and pre-existing intrinsic aortopathy. The changes are present since infancy and are more pronounced in older patients subjected to long-standing cyanosis and volume overload and may account for or may coexist with the higher incidence of aortic dilatation encountered in these patients.