Pediatric Heart Transplantation in the Context of Severe Pulmonary Hypertension Secondary to Restrictive Cardiomyopathy-Case Report.

The aim of this study is to analyze the feasibility of performing an isolated heart transplant in patients with severe pulmonary hypertension as a result of restrictive cardiomyopathy. The results present the clinical course from the diagnosis of restrictive cardiomyopathy at the age of 2 until the heart transplant at 8 years old. Initially, the patient was considered for multiorgan transplantation, heart and lungs, due to extremely high pulmonary resistance. However, due to the prolonged waiting period for a donor and the worsening condition of the child, a decision was made to perforate the atrial septum with the implantation of an atrial flow regulator system. After conducting control hemodynamic measurements, the qualification was changed to an isolated heart transplant, accepting the high operative risk associated with the still elevated pulmonary resistance index of 4.9 Wood units. This study describes the medical problems that occurred during postoperative treatment. The patient underwent an orthotopic heart transplant in her eighth year of life. Postsurgery, complications were observed, including generalized seizures and heart transplant rejection reaction. Immunosuppressive therapies were applied, and efforts were made to combat anemia and electrolyte disorders. While the cardiovascular system and heart parameters improved, there were some difficulties in controlling heart rhythm and stabilizing electrolyte levels.

Biventricular assist device implant using biatrial cannulation for restrictive cardiomyopathy.

Preoperative calculations showed that the 9-mm inlet, 6-mm outlet, 25-cc pump chambers and 65-73 bpm would be optimal for a 5-year-old patient suffering from restrictive cardiomyopathy, with a body surface area of 0.59 m2 (1.5 L/min flow for a cardiac index of 2.5). After re-sternotomy and standard bicaval cannulation for cardiopulmonary bypass, the procedure was performed under normothermic conditions and on the beating heart. Biventricular support was established with the Berlin Heart Excor using biatrial cannulation. For left atrial cannulation, induced ventricular fibrillation was used. The 9-mm inlet cannulas were inserted into the left and right atria, respectively. The 6-mm outlet cannulas were implanted using 8-mm interposition vascular grafts for the aorta and the main pulmonary artery, respectively. Cannulas were tunnelled through the epigastric space, with systems crossing outside of the body. The 25-cc chambers were used for both right ventricular assist device and left ventricular assist device support, which subsequently showed full emptying and filling.

Ventricular assist device support in paediatric patients with restrictive cardiomyopathy-clinical outcomes and haemodynamics.

OBJECTIVES: Restrictive cardiomyopathy is rare and is generally associated with worse clinical outcomes compared to other cardiomyopathies. Ventricular assist device (VAD) support for these children is seldom applied and often hampered by the surgical difficulties. METHODS: All paediatric (<19 years) patients with a restricted cardiomyopathy supported by a VAD from the EUROMACS database were included and compared to patients with a dilated cardiomyopathy (retrospective database analyses). Participating centres were retrospectively contacted to provide additional detailed echo and Swan Ganz measurements to analyse the effect of VAD support on pulmonary artery pressure and right ventricular function. RESULTS: Forty-four paediatric VAD-supported patients diagnosed with restricted cardiomyopathy were included, with a median age at implantation of 5.0 years. Twenty-six of the 44 patient with a restricted cardiomyopathy survived to transplantation (59.1%), 16 died (36.4%) and 2 are still on ongoing VAD support (4.5%) after a median duration of support of 95.5 days (interquartile range 33.3-217.8). Transplantation probability after 1 and 2 years of VAD support in patients with a restricted cardiomyopathy were comparable to patients with a dilated cardiomyopathy (52.3% vs 51.4% and 59.5% vs 60.1%, P = 0.868). However, mortality probability was higher in the restricted cardiomyopathy cohort (35.8% vs 17.0% and 35.8% vs 19.0%, P = 0.005). Adverse event rates were high (cerebrovascular accident in 31.8%, pump thrombosis in 29.5%, major bleeding 25.0%, eventual biventricular support in 59.1%). In the atrially cannulated group, cerebrovascular accident and pump thrombosis occurred in twice as much patients (21.1% vs 40.0%, P = 0.595 and 15.8% vs 40.0%, P = 0.464; probably non-significant due to the small numbers). Pulmonary arterial pressures improved after implantation of a VAD, and 6 patients who were initially labelled as ineligible due to pulmonary hypertension could eventually be transplanted. CONCLUSIONS: VAD support in children with a restricted cardiomyopathy is rarely performed. Mortality and adverse event rates are high. On the other hand, survival to cardiac transplantation was 59.1% with all patients surviving the 1st 30 days after cardiac transplantation. Pulmonary arterial pressures improved while on support, potentially making cardiac transplantation a viable option for previously ineligible children.

Cardiomiopatia restritiva idiopática / Idiopothic restrictive cardiomyopathy

PURPOSE--To characterize the idiopathic restrictive cardiomyopathy (RCM) through clinical manifestations and complementary tests and to analyze the surgical benefit on the correction of tricuspid regurgitation. METHODS--Five patients with RCM idiopathic were retrospectively studied, 4 female and 1 male, with ages ranging from 30 to 59 (mean 40) years. The patients presented heart failure due to restrictive syndrome and were submitted to chest X-ray, EKG, echocardiogram, right ventricular endomyocardial biopsy, hemodynamic study and surgery. RESULTS--X-ray showed heart enlargement in all patients, but only one had pulmonary venous capillary congestion. EKG presented atrial fibrillation, complex QRS with low voltage on frontal axis, abnormal ventricular repolarization, right bundle branch block, inactive area and right ventricular hypertrophy. Complete atrioventricular block was observed in one patient. The echocardiogram study showed biatrial enlargement in all patients, and left ventricular dilatation in one patient with left ventricular dysfunction. Mild tricuspid regurgitation were observed in one patient and moderate in four. Two patients had mild mitral regurgitation. Diastolic dysfunction were observed at Doppler-cardiography in all patients. Right ventricular endomyocardial biopsy showed interstitial fibrosis, myocytes hypertrophy and myofibrils disarray. The hemodynamic study disclosed right ventricular hypertension in all patients. Three patients had hypotension in left ventricle and aorta. The left ventricular end diastolic pressure and pulmonary capillary pressure (pulmonary wedge pressure) were elevated in all patients. Dilated left ventricle with mild hypokinesia and right ventricular inferior hypokinesia and diastolic restriction were observed in one patient. One patient had similar pattern without diastolic restriction. Mild tricuspid regurgitation was observed in one patient, moderate in three and severe in one. Mild mitral regurgitation were observed in two patients. The restrictive pattern (deep and plateau) was present in three patients. Surgery were performed in all patients, and confirmed the mild tricuspid regurgitation in one and the moderate in four. De Vega plastia were performed in four patients. CONCLUSION--Among the complementary methods, echocardiogram and hemodynamic study were those of higher contribution to the diagnosis of idiopathic RCM. The disease has a poor evolution, even with clinical or surgical treatment. Heart transplantation may would be the best procedure on patients with symptomatic idiopathic restrictive cardiomyopathy

Objetivo - Caracterizar a cardiomiopatia restritiva (CMR) idiopática através das manifestações clínicas e exames complementares e analisar o benefício da cirurgia de correção da insuficiência tricúspide (IT). Métodos - Foram estudados retrospectivamente cinco pacientes portadores de CMR idiopática, sendo quatro do sexo feminino e um do masculino, variando as idades entre 30 a 59 (média 40) anos. Os pacientes vieram encaminhados com síndrome restritiva e ICC e foram realizados radiografia de tórax, ECG, ecocardiograma, biópsia endomiocárdica de VD, estudo hemodinâmico e cirurgia. Resultados - A radiografia de tórax mostrou cardiomegalia em todos pacientes, sendo que um tinha congestão venocapilar pulmonar. Os ECG apresentavam fibrilação atrial, baixa voltagem de QRS em plano frontal, alteração da repolarização ventricular, bloqueio completo de ramo direito, área inativa e sobrecarga ventricular direita. Um paciente apresentou BAVT. O ecocardiograma mostrou aumento biatrial em todos pacientes, apenas um tinha dilatação ventricular esquerda com função sistólica diminuida. Havia IT discreta em 1 paciente, moderada em 4. Dois pacientes tinham insuficiência mitral (IM) discreta. Disfunção diastólica foi identificada nos 5 pacientes, através do estudo dopplercardiográfico. A biópsia de VD mostrou fibrose intersticial, hipertrofia de miócitos e desarranjo de miofibras. O estudo hemodinâmico mostrou hipertensão em câmaras direitas em todos pacientes. Três tinham hipotensão em VE e aorta. A Pd2VE e pressão capilar pulmonar estavam elevadas em todos pacientes. Um tinha VE dilatado com hipocinesia discreta, VD com hipocinesia inferior e restrição diastólica. Outro tinha achados semelhantes, porém sem restrição diastólica. IT discreta foi observada em 1 paciente, moderada em 3 e acentuada em 1. Dois pacientes tinham IM discreta. O padrão "depressão e platô " (sinal da "raiz quadrada ") estava presente em três pacientes. Foi realizada cirurgia nos 5 pacientes, sendo observada IT discreta em 1, moderada em 4 e realizada plastia de De Vega em 4. Conclusão - Dentre os exames complementares, o ecocardiograma e o estudo hemodinâmico são os que são os que mais contribuem para o diagnóstico de CRM idiopática. Os pacientes apresentaram evoluçào desconfortável mesmo com tratamento clínico e cirúrgico com correçào da insuficiência valvar. Por isso, consideramos o transplante cardíaco a conduta mais adequada para os pacientes com cardiomiopatia avançada