Jorge Yussef Afiune

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.

Anomalous subaortic course of the left brachiocephalic (innominate) vein: echocardiographic diagnosis and report of an unusual association

Subaortic left brachiocephalic, or innominate, vein is an uncommon finding in congenital heart disease, usually associated with obstruction of the right ventricular outflow tract. We describe our experience with 14 patients in whom the lesion was identified echocardiographically, 12 of them with right ventricular obstruction, one with totally anomalous pulmonary venous connection in the absence of obstruction to the right ventricular outflow tract, and the final one with a normal heart. A precise diagnosis of this venous anomaly is of great importance, since it needs to be differentiated from a central pulmonary artery, a pulmonary venous confluence, or an ascending vertical vein in totally anomalous pulmonary venous connection. In patients referred for surgery without catheterization, an incorrect echocardiographic diagnosis could lead to disastrous surgical results.

Emprego do suporte cardiopulmonar com bomba centrífuga e oxigenador de membrana em cirurgia cardiovascular pediátrica

BACKGROUND Extracorporeal membrane oxygenation is a well-documented resuscitation method in patients with severe hemodynamic and/or respiratory impairment. OBJECTIVE To describe several aspects related to the use of extracorporeal membrane oxygenation in a pediatric heart center and determine its immediate and late outcomes. METHODS Between October 2005 and January 2007, 10 patients who were submitted to pediatric cardiac surgery underwent extracorporeal membrane oxygenation implant. Median age was 58.5 days (40% neonates) and median body weight was 3.9 kg. Circulatory assistance was initiated aiming at the recovery and the weaning protocols followed daily clinical and echocardiographic criteria. Support was discontinued when transplant was contraindicated, when the patient was unable to recover or when survival was considered to be limited by a multidisciplinary team. RESULTS Extracorporeal membrane oxygenation was employed after corrective or palliative heart surgery in 80% and preoperatively in the remaining ones. It was most often indicated for irresponsive hemodynamic instability (40%), post-cardiotomy shock (20%) and post-cardiac arrest (20%). The mean duration on support was 58 +/- 37 hours. Weaning was successfully in 50% of the cases and 30% were discharged home. Actuarial survival was 40%, 30% and 20% at 30 days, 3 months and 24 months, respectively. CONCLUSION Extracorporeal membrane oxygenation is an effective and useful tool for the resuscitation of patients presenting severe hemodynamic and/or respiratory failure in the perioperative period of pediatric cardiovascular surgery.

Uma proposta para nomenclatura dos defeitos congênitos do sistema cardiovascular

The authors propose a new nomenclature system for congenital heart defects, based on the principles of the sequential segmental analysis. The short list of diagnosis is presented, and the importance of terminology uniformity is discussed, in order to facilitate the exchange of information among institutions.

Operação de Senning com a utilização de tecidos do próprio paciente

O grande atrativo da Operacao de Senning como descrita originalmente e a utilizacao das paredes do proprio atrio do paciente para a realizacao dos tuneis venosos. A atrioseptostomia por balao, utilizada atualmente na grande maioria dos casos de transposicao das grandes arterias (TGA), cria grandes comunicacoes interatriais. Com isso, e necessario a utilizacao de enxertos biologicos ou sinteticos (Dacron, Teflon e pericardio bovino) na septacao do atrio esquerdo. Doze criancas consecutivas, com idades de 5 meses a 4 anos (media = 20 meses), foram submetidas a Operacao de Senning com a utilizacao de tecidos do proprio paciente. O diagnostico era de TGA em 9, TGA com comunicacao interventricular (CIV) em 1, TGA com estenose pulmonar valvar discreta em 1, TGA com justaposicao das auriculas em 1 caso e todas as criancas foram submetidas a atrioseptostomia com balao no periodo neonatal. Em 5 casos foi utilizada a auricula esquerda aberta e invertida para a septacao interatrial, 1 com inversao e, em 5, inversao com abertura da auricula esquerda e uso do proprio pericardio in situ para a realizacao do tunel das veias pulmonares, devido as reduzidas dimensoes do atrio direito (1 caso de justaposicao das auriculas). O tempo de internacao variou de 10 a 24 dias (media 15 dias), sendo que o periodo de pos-operatorio variou de 7 a 22 dias (media 12 dias). Nao houve obitos no periodo de internacao hospitalar. O seguimento no pos-operatorio foi de 8 a 34 meses (media 23 meses) e todos os pacientes apresentaram boa evolucao clinica. O ecocardiograma realizado no periodo de internacao e no seguimento pos-operatorio nao evidenciou sinais de obstrucao ao fluxo nos tuneis intracardiacos. O uso de tecidos do proprio paciente in situ, com potencial possibilidade de crescimento, resgata a vantagem principal da tecnica originalmente descrita por Senning.

Atrioventricular septal defect with coexisting tricuspid atresia.

We describe two infants having an atrioventricular septal defect in the setting of a double inlet atrioventricular connection, but with patency of the left-sided valvar orifice and an imperforate right-sided valvar component, and a further case with atrioventricular septal defect and an imperforate Ebsteins malformation, all producing the haemodynamic effect of tricuspid atresia. We make comparisons with the arrangement in trisomy 16 mice, in whom deficient atrioventricular septation is seen at times with the common atrioventricular junction exclusively connected to the left ventricle, a situation similar to that seen in two of our infants. We also review previous reports emphasising the important theoretical implication of the findings despite their rarity.

Correção total da conexão anômala total das veias pulmonares em paciente adulto

A 22 year-old male patient was admitted with supracardiac, nonobstructive, total anomalous pulmonary venous connection and large atrial septal defect. Cardiac catheterization revealed severe pulmonary hypertension due to pulmonary overflow (Qp/Qs 6, pulmonary vascular resistance = 1.6 Woods/m(2)). Complete repair was performed through median sternotomy and cardiopulmonary bypass. The patient was discharged from hospital on the sixth postoperative day with no complications, using converting enzyme inhibitor and aspirin. At six-month follow-up he is currently asymptomatic, with preserved biventricular function and no echocardiographic signs of pulmonary hypertension.

Transvenous dual-chamber pacemaker after paediatric heart transplantation using left ventricle pacing through the coronary sinus

A 12‐year‐old child with end‐stage heart failure due to restrictive cardiomyopathy was submitted to orthotopic heart transplantation. Primary graft dysfunction required venous arterial extra‐corporeal membrane oxygenation. Heart function normalized, but complete atrioventricular block remained after 3 weeks. A dual‐chamber pacing with transvenous left ventricle pacing through the coronary sinus was performed. At 5‐year follow‐up, the patient is stable with the same pacing system and with preserved ventricular function.

Experiência inicial de um novo centro no Brasil na abordagem híbrida para a síndrome de hipoplasia do coração esquerdo

Hybrid Approach for Treatment of the Hypoplastic Left Heart Syndrome: Initial Experience of a New Center in Brazil Objective: Our objective was to review the early results of hybrid procedures in hypoplastic left heart syndrome (HLHS) at our institution. Methods: Eight HLHS patients were submitted to the first stage of the procedure and the indications were the surgeon’s preference in 4 (50%), sepsis in 2 (25%), severe ventricular dysfunction in 1 (12.5%) and severe brain damage with pulmonary hypertension in the latter (12.5%). Results: Three (37.5%) patients died after the intervention, 2 (25%) underwent the second stage and none underwent the third stage. Three (37.5%) patients are currently waiting for the second stage and 2 of these are currently hospitalized: one with pneumonia-related sepsis and the other with pneumonia post atrial septal defect stenting. Reinterventions were necessary in 50% of the cases, more than once in 2 patients (balloon atrioseptostomy in 3 and atrial septal defect stenting in 1). Mean mechanical ventilation duration in the first stage was 585 hours and mean hospitalization time in the intensive care unit was 32 days. Both patients undergoing the second stage died: sudden death in one and residual left pulmonary artery stenosis after percutaneous dilatation in the other. Conclusion: Hybrid procedures should be considered as complex as Norwood operation due to the learning curve, technically difficulties to perform the second stage, need of multiple interstage reinterventions and anatomic sequelae related to previous procedures.

Critical aortic stenosis in the neonate. Clinical assessment and surgical outcome in 18 patients

OBJECTIVE To assess neonates with aortic stenosis with early decompensation operated upon. (LCO) (CHF). METHODS A and retrospective study analyzing 6 neonates with LCO, group I (GI), and 12 neonates with CHF, group II (GII). Clinical radiographic, electrocardiographic and echocardiographic findings also provided comparative bases for the study, as did surgical and evolutional findings. RESULTS The mean ages at hospitalization and surgery (p = 0.0031) were 14.3 and 14.8 days in GI and 35.4 and 42.8 days in GII, respectively. Cardiac murmurs were more intense in GII (p = 0.0220). The aortic ring was smaller in GI (8.0 +/- 2.5mm) as compared to GII (11.4 +/ 1.4mm) (p = 0.2882). Ventricular function was reduced to 18 +/- 5.5% and 33.3 +/- 7.6% in GI and GII, respectively (p = 0.0162). Aortic atresia, however, was present only in 2 neonates in GI. Five of 6 patients in GI died but all patients in GII survived (p=0.0007). In the latter group, 84.6% of the patients were in functional class I (FC-I) in the long-term follow-up, with moderate residual lesions in 6 neonates, discrete residual lesions in 4, and reoperation in 2. CONCLUSION Aortic stenosis is a severe anomaly of the neonate, whose immediate evolution depends on the pre-operative anatomic and functional findings, and the late evolution essentially depends on the anatomic features of the valve.