Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection

Heart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. We report the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwent several prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technical modifications was performed successfully at the age of 19 year

Benefits of cardiac surgery in octogenarians — a postoperative quality of life assessment

Objective: Assessment of postoperative quality of life in patients over 80 years after cardiac surgery including coronary artery bypass grafting (CABG), aortic valve replacement (AVR) and combined procedures. Methods: Quality of life of n=136 patients over 80 years at operation (82.3±2.1 years), undergoing isolated CABG in 61 patients (45%), isolated AVR in 34 patients (25%) and a combination of CABG and AVR in 41 patients (30%) between January 1999 and December 2003 was reviewed. Preoperatively 66.2% presented in NYHA-class III/IV or CCS-class III/IV. Mean ejection fraction (EF) was 59.5%±14.0 (range 25-90%). Quality of life assessment was performed via a Seattle Angina Questionnaire. Follow-up was 100% complete for a total of 890 days (69-1853 days). Results: Five-year survival was 70% for the CABG group, 75% for the AVR group and 65% for the CABG/AVR group. Quality of life was remarkable in all of the three groups after surgery. Overall 97 patients (81%) were not or little disabled in their daily activity. One hundred and twelve patients (93%) were free or considerably less symptomatic. Seventy-eight patients or 65% reported to be very satisfied with their current quality of life and 112 patients (93%) felt very reassured to have continuous full access to medical treatment despite of their advanced age. Conclusions: A remarkable quality of life and important improvement in the functional status after cardiac surgery in patients over 80 paired with a satisfactory medium-term survival justify early intervention for heart disease in this age group. Therefore, referral practice for patients over 80 years for heart surgery should be handled liberall

Impact of clopidogrel in coronary artery bypass grafting

Objective: Clopidogrel has become the standard of care to prevent thrombotic complications following cardiological interventions, in particular intracoronary stenting. In addition, patients with aspirin intolerance and those with carotid and peripheral vascular disease are also increasingly treated with clopidogrel. Platelet inhibition may become a concern for hemostasis in patients treated with clopidogrel who need emergency and undelayed surgery. Methods: We prospectively analyzed the intra- and postoperative outcome of 505 consecutive patients who underwent isolated CABG and compared two groups: those with clopidogrel exposure until 72 h prior to surgery (n=136) with those without exposition to clopidogrel (n=369). Patients undergoing emergency surgery because of failed PTCA and cardiogenic shock, associated valvular surgery, redo-CABG, and those with additional platelet IIb/IIIa receptor inhibitor exposure were excluded. Patients who received aspirin and/or heparin treatment prior to surgery were not excluded. Results: Patients who received clopidogrel had a higher prevalence of angina class III or IV (67 vs 39%, P<0.01), received more often revascularization within 48 h (41 vs 14%, P=0.02), and had received more frequently stenting (57 vs 13%). Chest tube drainage was significantly increased during the first 24 h following CABG in the group of patients who had clopidogrel treatment (1485 vs 780 ml, P=0.003) These patients also required more transfusion of platelets and fresh frozen plasma. Overall re-exploration rate because of bleeding was significantly higher in the clopidogrel group (5.9 vs 1.2%, P<0.01). Platelets transfused before chest closure had a beneficial effect on preservation of the hemostasis. Conclusions: Clopidogrel exposure 3 days or less prior to CABG surgery significantly increases the risk of postoperative bleeding, the need for perioperative transfusion and the incidence of re-exploration. Surgery should be performed using standard heparinization and anti-fibrinolytic strategies but aggressive correction of platelets dysfunction is required before chest closur

Addition of dextran sulfate to blood cardioplegia attenuates reperfusion injury in a porcine model of cardiopulmonary bypass

Objective: Contact of blood with artificial surfaces and air as well as ischemia/reperfusion injury to the heart and lungs mediate systemic and local inflammation during cardiopulmonary bypass (CPB). Activation of complement and coagulation cascades leads to and accompanies endothelial cell damage. Therefore, endothelial-targeted cytoprotection with the complement inhibitor and endothelial protectant dextran sulfate (DXS, MW 5000) may attenuate CBP-associated myocardial and pulmonary injury. Methods: Eighteen pigs (DXS, n=10; phosphate buffered saline [PBS], n=8) underwent standard cardiopulmonary bypass. After aortic cross-clamping, cardiac arrest was initiated with modified Buckberg blood cardioplegia (BCP), repeated after 30 and 60min with BCP containing either DXS (300mg/10ml, equivalent to 5mg/kg) or 10ml of PBS. Following 30min reperfusion, pigs were weaned from CPB. During 2h of observation, cardiac function was monitored by echocardiography and invasive pressure measurements. Inflammatory and coagulation markers were assessed regularly. Animals were then sacrificed and heart and lungs analyzed. Results: DXS significantly reduced CK-MB levels (43.4±14.8ng/ml PBS, 35.9±11.1ng/ml DXS, p=0.042) and significantly diminished cytokine release: TNFalpha (1507.6±269.2pg/ml PBS, 222.1±125.6pg/ml DXS, p=0.0071), IL1beta (1081.8±203.0pg/ml PBS, 110.7±79.4pg/ml DXS, p=0.0071), IL-6 (173.0±91.5pg/ml PBS, 40.8±19.4pg/ml DXS, p=0.002) and IL-8 (304.6±81.3pg/ml PBS, 25.4±14.2pg/ml DXS, p=0.0071). Tissue endothelin-1 levels were significantly reduced (6.29±1.90pg/100mg PBS, 3.55±1.15pg/100mg DXS p=0.030) as well as thrombin-anti-thrombin formation (20.7±1.0μg/ml PBS, 12.8±4.1μg/ml DXS, p=0.043). Also DXS reduced cardiac and pulmonary complement deposition, neutrophil infiltration, hemorrhage and pulmonary edema (measured as lung water content, 81±3% vs 78±3%, p=0.047), indicative of attenuated myocardial and pulmonary CPB-injury. Diastolic left ventricular function (measured as dp/dtmin), pulmonary artery pressure (21±3mmHg PBS, 19±3mmHg DXS, p=0.002) and right ventricular pressure (21±1mmHg PBS, 19±3mmHg DXS p=0.021) were significantly improved with the use of DXS. Conclusions: Addition of DXS to the BCP solution ameliorates post-CPB injury and to a certain extent improves cardiopulmonary function. Endothelial protection in addition to myocyte protection may improve post-CPB outcome and recover

Surgery of the dilated aortic root and ascending aorta in pediatric patients: techniques and results

Objective: Dilatation of the aortic root is a well-known cardiovascular manifestation in children and adult patients with connective tissue disease (e.g. Marfan syndrome). Dilatation of the ascending aorta is extremely rare and may be associated with bicuspid aortic valve. This report evaluates the incidence of dilatative aortic root and ascending aortic pathology in patients younger than 18 years and analyzes the results obtained after repair and replacement strategies. Methods: Between 1/1995 and 12/2002, a total of 752 operations on the thoracic aorta were performed in adult and pediatric patients. We present our experience with a group of 26 patients <18 years of age, who required isolated surgery of the aortic root and/or ascending aorta because of a dilatative lesion. Fifteen patients had isolated aortic root dilatation (13 of them suffered from Marfan syndrome), eight patients presented with an idiopathic dilatation of the ascending aorta and three patients had dilatation in association with a bicuspid aortic valve. Mean age was 10±4.8 years (4-18 years). Repair of the aortic root with preservation of the aortic valve (Yacoub, David or selective sinus repair) was performed in nine patients, replacement using a homograft was performed in five patients, composite graft with mechanical prosthesis in two patients, with biological prosthesis in one patient and Ross operation was performed in one case. Isolated supracoronary graft replacement was performed in eight patients. Results: Two patients died during hospitalization: a 10-year old girl developed respiratory failure on the 2nd postoperative day and autopsy revealed Ehlers-Danlos syndrome with a massive intrapulmonary emphysema. A 14-year-old Marfan patient with severely depressed preoperative LV function died from low cardiac output following composite-graft, mitral and tricuspid valve repair. One patient required aortic valve replacement 7 days after an aortic valve sparing root repair. There was no additional perioperative morbidity. In the long-term, two patients died from rupture of the thoracic aorta, both following minor non-cardiovascular surgical procedures. Both had normal sized descending and abdominal aorta. Conclusion: Repair of the aortic root and/or ascending aorta in children and adolescent patients can be performed with acceptable early and late results. While the presence of severe comorbidity may adversely affect early outcome, long-term survival was mainly determined by rupture of the descending aort

Failed coronary artery bypass anastomosis detected by intraoperative coronary flow measurement

Objectives: To assess intraoperative flow of arterial and venous coronary grafts after myocardial revascularization which may allow early detection of low flow situations, especially during minimally invasive coronary bypass surgery (MIDCAB), and lead to immediate correction of technical problems. Methods: In two patients with severe and diffuse multi-vessel disease the left internal mammary artery (IMA) was connected to the left anterior descending artery (LAD). During reperfusion, the flow was measured in the IMA and vein grafts using a transit time flow meter. Results: In both cases the IMA showed only a systolic pendulating flow curve with a mean flow of 0-1 ml/min and a high resistance. Manual IMA assessment revealed an adequate pulsation. Both distal IMA anastomoses were re-explored on cardiopulmonary bypass yielding an initial flow of 7 and 14 ml/min, respectively. After treatment with papaverine/adenosine the IMA flow increased from 7 to 26 ml/min (coronary flow reserve (CFR)=3.7) and from 14 to 46 ml/min (CFR=3.3), respectively. Conclusion: Intraoperative flow assessment of IMA and venous bypass grafts can be recommended to monitor flow; especially during MIDCAB procedure

Risk factors for secondary dilatation of the aorta after acute type A aortic dissection

Objectives: Prompt diagnosis of subsequent dilatation of the dissected aorta is crucial to reduce late mortality in these patients. This study focuses on risk factors for dilatation of the aorta after type A aortic dissection (AADA) affecting a normal-sized or slightly dilated aorta. Methods: Overall 531 CT scans were analysed. Patients were included in the study if at least 3 CT scans were available after operative repair. 64 patients (59.8%) out of 107 patients full-field the inclusion criteria. Volumetric analyses of the aorta were performed. Patients were divided in 3 groups: group A included 26 patients (40.6%) without progression of the aortic diameter, group 2, 27 patients (42.2%) with slight progression and group 3, 11 patients (17.2%) with important progression, requiring surgery in 9 patients (81.8%). Risk-factors for progression of the aortic size were analysed and compared between the groups. Results: Patients from group 3 were younger 57.7±13.4 vs. 61.9±11.6 in group 1 (P≪0.05) and were more frequent female (45.4 vs. 23.1%; P≪0.05). Dissection of the supraaortic branches (100 vs. 80.8%; P≪0.05), the presence of preoperative cerebral, visceral or peripheral malperfusion (54.6 vs. 26.9%; P≪0.05) and contrast enhancement in the false lumen during the follow-up (72.7 vs. 57.7%; P=0.07) were additional risk factors for late aortic dilatation in these patients. Conclusions: Acute type A aortic dissection in younger patients, involving the supraaortic branches and/or combined with malperfusion syndrome favour secondary dilatation. A close follow-up is mandatory to prevent acute complications of the diseased downstream aorta following repair of a AAD

Risk factors for secondary dilatation of the aorta after acute type A aortic dissection

Objectives: Prompt diagnosis of subsequent dilatation of the dissected aorta is crucial to reduce late mortality in these patients. This study focuses on risk factors for dilatation of the aorta after type A aortic dissection (AADA) affecting a normal-sized or slightly dilated aorta. Methods: Overall 531 CT scans were analysed. Patients were included in the study if at least 3 CT scans were available after operative repair. 64 patients (59.8%) out of 107 patients full-field the inclusion criteria. Volumetric analyses of the aorta were performed. Patients were divided in 3 groups: group A included 26 patients (40.6%) without progression of the aortic diameter, group 2, 27 patients (42.2%) with slight progression and group 3, 11 patients (17.2%) with important progression, requiring surgery in 9 patients (81.8%). Risk-factors for progression of the aortic size were analysed and compared between the groups. Results: Patients from group 3 were younger 57.7±13.4 vs. 61.9±11.6 in group 1 (P≪0.05) and were more frequent female (45.4 vs. 23.1%; P≪0.05). Dissection of the supraaortic branches (100 vs. 80.8%; P≪0.05), the presence of preoperative cerebral, visceral or peripheral malperfusion (54.6 vs. 26.9%; P≪0.05) and contrast enhancement in the false lumen during the follow-up (72.7 vs. 57.7%; P=0.07) were additional risk factors for late aortic dilatation in these patients. Conclusions: Acute type A aortic dissection in younger patients, involving the supraaortic branches and/or combined with malperfusion syndrome favour secondary dilatation. A close follow-up is mandatory to prevent acute complications of the diseased downstream aorta following repair of a AADA

Addition of dextran sulfate to blood cardioplegia attenuates reperfusion injury in a porcine model of cardiopulmonary bypass

OBJECTIVE: Contact of blood with artificial surfaces and air as well as ischemia/reperfusion injury to the heart and lungs mediate systemic and local inflammation during cardiopulmonary bypass (CPB). Activation of complement and coagulation cascades leads to and accompanies endothelial cell damage. Therefore, endothelial-targeted cytoprotection with the complement inhibitor and endothelial protectant dextran sulfate (DXS, MW 5000) may attenuate CBP-associated myocardial and pulmonary injury. METHODS: Eighteen pigs (DXS, n=10; phosphate buffered saline [PBS], n=8) underwent standard cardiopulmonary bypass. After aortic cross-clamping, cardiac arrest was initiated with modified Buckberg blood cardioplegia (BCP), repeated after 30 and 60 min with BCP containing either DXS (300 mg/10 ml, equivalent to 5mg/kg) or 10 ml of PBS. Following 30 min reperfusion, pigs were weaned from CPB. During 2h of observation, cardiac function was monitored by echocardiography and invasive pressure measurements. Inflammatory and coagulation markers were assessed regularly. Animals were then sacrificed and heart and lungs analyzed. RESULTS: DXS significantly reduced CK-MB levels (43.4+/-14.8 ng/ml PBS, 35.9+/-11.1 ng/ml DXS, p=0.042) and significantly diminished cytokine release: TNFalpha (1507.6+/-269.2 pg/ml PBS, 222.1+/-125.6 pg/ml DXS, p=0.0071), IL1beta (1081.8+/-203.0 pg/ml PBS, 110.7+/-79.4 pg/ml DXS, p=0.0071), IL-6 (173.0+/-91.5 pg/ml PBS, 40.8+/-19.4 pg/ml DXS, p=0.002) and IL-8 (304.6+/-81.3 pg/ml PBS, 25.4+/-14.2 pg/ml DXS, p=0.0071). Tissue endothelin-1 levels were significantly reduced (6.29+/-1.90 pg/100mg PBS, 3.55+/-1.15 pg/100mg DXS p=0.030) as well as thrombin-anti-thrombin formation (20.7+/-1.0 microg/ml PBS, 12.8+/-4.1 microg/ml DXS, p=0.043). Also DXS reduced cardiac and pulmonary complement deposition, neutrophil infiltration, hemorrhage and pulmonary edema (measured as lung water content, 81+/-3% vs 78+/-3%, p=0.047), indicative of attenuated myocardial and pulmonary CPB-injury. Diastolic left ventricular function (measured as dp/dt(min)), pulmonary artery pressure (21+/-3 mmHg PBS, 19+/-3 mmHg DXS, p=0.002) and right ventricular pressure (21+/-1 mmHg PBS, 19+/-3 mmHg DXS p=0.021) were significantly improved with the use of DXS. CONCLUSIONS: Addition of DXS to the BCP solution ameliorates post-CPB injury and to a certain extent improves cardiopulmonary function. Endothelial protection in addition to myocyte protection may improve post-CPB outcome and recovery

Successful heart transplantation in a patient with Ivemark syndrome combined with situs inversus, single atrium and ventricle after total cavo-pulmonary connection

Heart transplantation represents a valuable therapeutical option for patients with congenital heart disease and end-stage heart failure. We report the case of a young adult patient with a situs inversus and additional complex congenital malformations of the heart who underwent several prior palliative interventions, a biventricular repair being impossible. Orthotopic cardiac transplantation with several technical modifications was performed successfully at the age of 19 years