José Henrique Andrade Vila

Anomalia de Ebstein: resultados com a reconstrução cônica da valva tricúspide

OBJECTIVE: To assess the results of a technical modification of tricuspid valvuloplasty in Ebsteins anomaly. METHODS: From November 1993 to August 2002, 21 patients with Ebsteins anomaly of the tricuspid valve, with ages ranging from 20 months to 37 years (mean, 23 years), underwent a new technique of tricuspid valvuloplasty. This technique consisted of total or almost total detachment of the anterior tricuspid megaleaflet from the ventricular wall and valvular ring, transforming it into a cone, whose vertex remained fixed in the right ventricular tip, and the base was sutured to the tricuspid ring, after its plication, adjusting it to the size of the base of the cone (tricuspid ring), including the septal region. RESULTS: One (4.7%) patient with cardiomyopathy caused by chronic hypoxia died in the hospital due to low cardiac output. The mean follow-up lasted 4 years, and the recent echocardiograms showed good morphology of the right ventricle and tricuspid valve with mild or minimal insufficiency in 18 patients and moderate insufficiency in 2 patients. In 2 of the 3 patients with an anomalous bundle, it could be located and sectioned during surgery. No atrioventricular block occurred. CONCLUSION: The technique used was efficient in repairing tricuspid insufficiency and restoring right ventricular morphology, being applicable to all anatomic types of Ebsteins anomaly, except for Carpentier classification type D.

Síndrome do coração esquerdo hipoplásico: estratégia cirúrgica e comparação de resultados com técnicas de Norwood x Sano

Objectives: To report a surgical strategy for the Norwood procedure in hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest and aortic arch reconstruction using autologous pericardium. Additionally, the work compares the results of the modified BlalockTaussig (mBT) shunt and the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow. Method: A retrospective study was performed of 78 newborns consecutively operated on between March 1999 and June 2006. One technique to reconstruct the neoaorta and two different techniques, to reestablish the pulmonary blood flow, were utilized - mBT shunts in the first 37 newborns and RV-PA conduits in the last 41. Cannulation of the ductus arteriosus for systemic perfusion was the main part of the surgical strategy used to reduce the hypothermic circulatory arrest time. Results: In-hospital survival for the entire cohort was 74.35%, or 67.57% for the mBT shunt and 80.49% for the RVPA conduit Groups (p=0.21). Hypothermic circulatory arrest times were 45.79 ± 1.99 minutes and 36.8 ± 1.52 minutes (p=0.0012), respectively. Mortality rates between first and second stages were 40% for the mBT shunt and 6.9% for the RV-PA conduit Groups (p=0.007). Late coarctation of the aorta occurred in five patients. A comparison of the actuarial survival curves (Kaplan-Meier) showed that the results were better with the VD-AP conduit (p=0.003). Conclusions: This surgical strategy resulted in a short circulatory arrest time, low mortality and a low incidence of aortic coarctation. Although the higher rate of survival in the first palliation stage with the RV-PA conduit was not significant, the lower interstage mortality and superior medium-term survival in the RV-AP Group were statistically advantageous.

Síndrome da hipoplasia do coração esquerdo: a influência da estratégia cirúrgica nos resultados

OBJECTIVES To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch, and to compare the results of the modified Blalock-Taussig (mBT) shunt with the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow. METHODS Retrospective study of 71 newborns with HLHS consecutively operated between March, 1999 and February, 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow were used: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 34. Cannulation of the ductus arteriosus for arterial perfusion was the main part of the surgical strategy to reduce the hypothermic circulatory arrest time. RESULTS In-hospital survival for the entire cohort was 74.64%, or 67.57% and 82.35% for the mBT shunt and RV-PA conduit groups, respectively (p=0.1808). Mortality rates between the first and second palliation stages were 40% and 4.4% for the mBT shunt and RV-PA conduit groups, respectively (p=0.0054). Hypothermic circulatory arrest times were 45.79+/-1.99 min and 36.62+/-1.62 min (p=0.0012), respectively. Late coarctation of the aorta occurred in five patients (7.2%). CONCLUSION This surgical strategy resulted in short circulatory arrest time, low mortality and favorable morphology of the neoaorta, with low incidence of late coarctation of the aorta. The higher rate of survival to first palliation stage with the RV-PA conduit was not significant, but interstage mortality was statistically lower when compared with the modified Blalock-Taussig shunt procedure.

Cardiogenic shock due to citomegalovirus myocarditis: successful clinical treatment

OBJECTIVE Cytomegalovirus (CMV) systemic disease and myocarditis in healthy persons is infrequently reported in the literature, although in increasing numbers in recent years. The importance of the recognition of the syndrome that usually has an initial picture of a mononucleosis like infection in an otherwise healthy person, is the available therapeutic agent, ganciclovir, that can cure the infectious disease. METHODS We analyzed the clinical result of pulsotherapy with steroids in a patient with CMV myocarditis after 7 days of etiological treatment, with ganciclovir, intravenous vasodilators, and the conventional treatment for congestive heart failure. RESULTS The clinical condition of the patient improved accordingly to the better function of the left ventricle, and the ganciclovir was kept for 21 days, most of it in an out patient basis. The patient was dismissed from the hospital, with normal myocardial function. CONCLUSION Potentially curable forms of myocarditis, like M pneumoniae and CMV, for example, can have an initial disproportionate aggression to the myocardium, by the acute inflammatory reaction, that can by itself make worse the damage to the LV function. In our opinion, the blockade of this process by pulsotherapy with steroids can help in the treatment of these patients. We understand that the different scenario of immunosuppressive treatments for the possible auto immunity of the more chronic forms of the presumably post viral cardiomyopathy has been in dispute in the literature, and has stolen the focus from the truly acute cases.

Transplante cardíaco em amiloidose primária

Cardiac amyloidosis is a disease that highly compromises the survival expectancy after the beginning of the symptomatic phase, usually with sudden death as the final event. The aggression to other organs, although, can make heart transplantation a disputable form of treatment taking into consideration the shortage of organ donors. The aim is to report the evolution with a survival of seven years after heart transplantation and in fair condition of a patient with amyloidosis. One year after the heart transplantation, the patient was referred to renal transplantation also in consequence of the disease aggression. The patient evolution was favorable compared to three other patients also from our service, who died early after the diagnosis. Even considering the multi-systemic nature of amyloidosis, we can accept that in selected patients the heart transplantation is justified, taking into account the very ill prognosis of the disease.

A 23 years of uneventful evolution in a heart-transplanted patient with chagasic cardiomyopathy on a two-drug immunosuppressive protocol.

Address correspondence to: Lionel Rostaing, M.D., Ph.D., Department of Nephrology, Dialysis and Multiorgan Transplantation, Toulouse University Hospital, 1 Avenue Jean Poulhès, TSA 50032, 31059 Toulouse Cédex 9, France. E-mail: rostaing.l@chu-toulouse.fr Received 19 September 2008. Accepted 29 October 2008. Copyright

Experience with heterotopic heart transplantation in patients with elevated pulmonary vascular resistance: late follow-up

BACKGROUND Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5 WU, with a transpulmonary gradient above 15 mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS One immediate death occurred from graft failure, one death occurred after 2 (1/2) years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patients 15-year survival has demonstrated a long-term effectiveness of this option for selected patients.BACKGROUND: Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE: To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS: The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5WU, with a transpulmonary gradient above 15mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS: One immediate death occurred from graft failure, one death occurred after 2 ½ years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION: Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patients 15-year survival has demonstrated a long-term effectiveness of this option for selected patients.

Experiência com transplante cardíaco heterotópico em pacientes com resistência pulmonar elevada: seguimento tardio

BACKGROUND Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5 WU, with a transpulmonary gradient above 15 mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS One immediate death occurred from graft failure, one death occurred after 2 (1/2) years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patients 15-year survival has demonstrated a long-term effectiveness of this option for selected patients.BACKGROUND: Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE: To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS: The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5WU, with a transpulmonary gradient above 15mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS: One immediate death occurred from graft failure, one death occurred after 2 ½ years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION: Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patients 15-year survival has demonstrated a long-term effectiveness of this option for selected patients.

Experiencia con trasplante cardíaco heterotópico en pacientes con resistencia pulmonar elevada: seguimiento tardío

BACKGROUND Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5 WU, with a transpulmonary gradient above 15 mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS One immediate death occurred from graft failure, one death occurred after 2 (1/2) years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patients 15-year survival has demonstrated a long-term effectiveness of this option for selected patients.BACKGROUND: Along the past few years the number of papers on heterotopic cardiac transplant has been very scarce in the medical literature, including at the international level; this is particularly true in reference to the long term follow-up of these patients and the reason which led to the presentation of our report. OBJECTIVE: To report the initial clinical experience and late evolution of 4 patients undergoing heterotopic heart transplantation, indications for this procedure and its major complications. METHODS: The surgeries were performed between 1992 and 2001, and all had as indication for heterotopic transplantation the PVR, which ranged from 4.8 WU to 6.5WU, with a transpulmonary gradient above 15mmHg. In the 3rd patient, a direct anastomosis between the pulmonary arteries was performed without the use of a prostetic tube, and a mitral valvuloplasty and a LV aneurysmectomy were performed in the native heart. The immediate immunosuppressive regimens were double, with cyclosporine and azathioprine in the first 3 patients, and cyclosporine and mycophenolate mofetil in the 4th patient. RESULTS: One immediate death occurred from graft failure, one death occurred after 2 ½ years, from endocarditis in an intraventricular thrombus in the native heart, and a third death occurred 6 years after transplantation, from post-operative complications of the aortic valve surgery in the native heart. The remaining patient is well, 15 years after the transplantation. This patient is in functional class II (NYHA), 6 years after a surgical occlusion of the native heart aortic valve. CONCLUSION: Heterotopic heart transplantation results are inferior to those of orthotopic heart transplantation because they present higher RVP. The intraventricular thrombi, in the native heart, which require prolonged anticoagulation, and aortic valve complications, also in the native heart, may require surgical treatment. However, a patients 15-year survival has demonstrated a long-term effectiveness of this option for selected patients.

Late results of pulmonary root translocation in the correction of transposition of the great arteries

OBJECTIVE: The purpose of this study was to evaluate the late follow-up of pulmonary root translocation, a technique that aims to avoid complications and the need of reoperations related to the Rastelli procedure, in the repair of ventriculo-arterial connection anomalies associated to pulmonary stenosis and ventricular septal defect. METHODS: Five patients, ranging from two months to three years of age, were submitted to anterior pulmonary root translocation, from April 1994 to July 1999. The surgical technique consisted of pulmonary root removal from the left ventricle and its connection to the right ventricle, after patch diversion of the blood flow from the left ventricle to the aorta, through the ventricular septal defect. The construction of the right ventricle outflow tract was completed with autologous pericardium. RESULTS: There were neither early nor late deaths. There was no need for re-intervention. The late clinical and echocardiographic follow-ups showed some growth of the pulmonary root, with no clinically significant transpulmonary valve gradient (zero to 41 mmHg) and all patients were asymptomatic. CONCLUSION: Pulmonary root translocation showed to be efficient in the repair of ventriculo-arterial connection anomalies, with ventricular septal defect and pulmonary stenosis, and it was employed even in small children, with good early and long-term results, without the need for late reoperations in this small group of patients.