Transvascular Implantation of an Implantable Cardioverter-Defibrillator in a Patient Who has Undergone One-and-a-Half Ventricle Repair

Implantable cardioverter-defibrillator (ICD) therapy is acknowledged as a valid treatment method for the effective prevention of sudden cardiac death, which is a major cause of mortality in adult congenital heart disease patients. But ICD implantation by the conventional transvascular approach is not always possible in patients who have undergone palliative surgery due to congenital and structural heart disease. Here, we report a case in which an ICD was transvascularly implanted in an arrhythmogenic right ventricular cardiomyopathy patient who had undergone a one-and-a-half ventricle repair.

Morbilidad y mortalidad posoperatoria en niños con corazón univentricular según diagnóstico morfológico / Postoperative mortality and morbidity found in children with univentricular heart according to the morphological diagnosis

Introducción: la atresia tricuspídea es la cardiopatía congénita cianótica que motiva mayor indicación de cirugía univentricular. El tratamiento quirúrgico consiste en separar las circulaciones sistémica y pulmonar a través de una derivación cavopulmonar total (proceder de Fontan). Objetivo: comparar la evolución posoperatoria del proceder de Fontan entre enero de 1990 y diciembre de 2010 en relación con el diagnóstico morfológico. Métodos: se ordenaron en 2 grupos de estudio (AT: atresia tricuspídea y No AT: con diagnóstico diferente a atresia tricuspídea). Las variables cualitativas y cuantitativas se analizaron a través de las pruebas chi cuadrado y t de student para un nivel de significación £ 0,05. Resultados: presentaron atresia tricuspídea 31 pacientes y 61 no atresia tricuspídea. En el posoperatorio 68 pacientes presentaron complicaciones, 23 con atresia tricúspidea (74,2 por ciento) y 45 con diagnóstico diferente a atresia tricuspídea (73,7 por ciento). Treinta pacientes presentaron efusiones pleurales persistentes durante el posoperatorio, 11 (35,5 por ciento) y 19 (31,4 por ciento) para la atresia tricúspidea y con diagnóstico diferente a atresia tricuspídea respectivamente. Fallecieron 18 enfermos, sin diferencia significativa entre ambos grupos. Durante el seguimiento, la causa de morbilidad más frecuentes fue por capacidad funcional disminuida de forma moderada o grave, en 13 pacientes con atresia tricúspidea (44,8 `por ciento) y en 20 (44,4 por ciento) en el otro grupo (p= 1). Conclusiones: el proceder de Fontan es una opción quirúrgica que permite la supervivencia de los pacientes con corazón univentricular asociada a morbilidad posoperatoria a largo plazo. El diagnóstico morfológico de atresia tricuspídea no se relaciona con la morbilidad y mortalidad

Introduction: tricuspid atresia is the cyanotic congenital heart disease that more frequently involves the indication of univentricular heart surgery. The surgical treatment consists of separating the systemic circulation from the pulmonary one through total cavopulmonary shunt (Fontan procedure). Objective: to compare the evolution of Fontan procedure postoperatively from January 1990 to December 2010 with the morphological diagnosis. Methods: the children were divided into 2 study groups (Triscupid atresia and Triscupid atresia-free with diagnosis different from that of tricuspid atresia). Chi square and Student's tests helped to analyze qualitative and quantitative variables for significance level £ 0.05. Results: tricuspid atresia was observed in 31 patients and 61 tricuspid atresia-free. In the postoperative period, 68 patients suffered complications, 23 with tricuspid atresia (74.2 percent) and 45 diagnosed with a disease other than tricuspid atresia (73.7 percent). Thirty patients had persistent pleural effusions during the postoperative period, 11 (35.5 percent) and 19 (31.4) in the tricuspid atresia group and in the tricuspid atresia-free group respectively. Eighteen patients died and no significant association was found in neither of the groups. During the follow-up, the most frequent cause of morbidity was reduced functional capacity either moderate or severe in 13 patients with tricuspid atresia (44.8 percent) and in 20 children (44.4 percent)from the other group (p= 1). Conclusions: Fontan procedure is a surgical option that allows the survival of patients with univentricular heart associated to long-term postoperative morbidity. The morphological diagnosis of tricuspid atresia is not related to morbility and mortality

Advantages and disadvantages of bidirectional cavopulmonary shunt with additional pulmonary blood flow : a theoretical analysis / 中华胸心血管外科杂志

Objective The bidirectional cavopulmonary shunt (BCPS) is a major step in the staged palliation of functionally univentricular heart defects.Whether to preserve of additional pulmonary blood flow (APBF) has been a highly controversial issue.The purpose is to mathematical model and set out to determine the significa advantages and disadvantages of bidirectional cavopulmonary shunt with additional pulmonary blood flow:a theoretical analysis nce of APBF and the appropriate APBF ratio.Methods We used models of the univentricular circulation after the bidirectional cavopulmonary anastomosis with additional pulmonary blood and to computational analyze the impact of APBF on oxygen delivery,APBF flow on the CVP and SaO2.Results The influence of APBF depends on the ratio of superior vena cava flow to inferior vena cava flow ( QSVC/QIVC ).For QSVC/QIVC ＞ 0.3,APBF may be associated with decreased oxygen delivery.For QSVC/QIVC ＜ 0.3,appropriate APBF may be associated with increased oxygen delivery.A linear relationship exists between the increase of APBF and CVP,and the slope was depended on the value of pulmonary vascular resistance.Estimating APBF from CVP measurements may be a feasible method.A nonlinear relationship between the increase of APBF and oxygen saturation,and estimating APBF from SaO2 measurements may result in errors.BCPS and appropriate APBF may optimal the oxygen delivery with the increase of age and the decrease in QSVC/QIVC.For patients who accepted BCPS without APBF,there is a decreasing tendency of oxygen delivery with the increase of age and the decrease in QSVC/QIVC.For patients who suffered pulmonary arterivenous malformation,there is a more obvious decrease in oxygen delivery.ConclusionFor patients under age who has normal pulmonary vascularbed ( that is,QSVC/QVC ＞ 0.3),elimination of additional pulmonary blood flow can improve the oxygen delivery under a given cardiac output.For patients who with hypoplastic pulmonary vascular or in older patients under age,APBF is necessary to improve oxygen delivery.For patients who have to accept BCPS as the final procedure,preserving of APBF is suggested.

Hetzer Technique for Surgical Correction of Ebstein's Anomaly / 대한흉부외과학회지

BACKGROUND: The Hetzer procedure for the correction of Ebstein's anomaly has the advantages of technical feasibility and incorporation of the atrialized right ventricle (RV) into the functional RV. MATERIAL AND METHOD: We preformed a retrospective review of 11 patients with Ebstein's anomaly and they had undergone a Hetzer procedure between March 2002 and December 2006. RESULT: The median age at operation was 19.8 years (range: 6 months~56 years). There were 4 males and 7 females. All patients showed severe tricuspid regurgitation (TR) preoperatively, and arterial desaturation (<95%) was present in 3 patients. The original Hetzer technique was employed in 6 patients with the Carpentier type A anomaly. In the remaining 5 patients with the Carpentier type B or C anomalies, valve competence was restored at the level of the displaced tricuspid valve mechanism. Adjunct bidirectional cavo-pulmonary shunt, or one and a half ventricle repair strategy was employed for all the patients. The median follow-up was 8.6 months (range: 0.8~51.9 months). There was no early or late death, and the immediate postoperative TR was trivial to mild in 8 patients. The median cardio-thoracic ratios on chest X-ray at the preoperative period and at postoperative 0, 1 and 6 months were 65%, 62%, 55% and 55%, respectively. CONCLUSION: The original or modified Hetzer procedure for Ebstein's anomaly shows excellent intermediate-term outcomes.

The repair of Ebstein's anomaly with ventricular unloading / 中华胸心血管外科杂志

Objective To summarize experiences in surgical treatments of 18 patients suffered from Ebstein's anomaly with intracardiac repair and bi-directional cavopulmonary shunt from January 1999 to June 2003. Methods Of 18 patients, 10 male (55.6%) and 8 female (44.4%), mean age was (18.47?13.85) years old (range from 9 months～54 years old),and mean weight of body (36.09?19.78) kg (range from 8.5～80.0 kg). All patients were in type II of Ebstein's anomaly according to WANG (Zenwei's) classification, while 15 (83.3%) were in type B, and 3 (16.7%) in type C according to Carpentier's classification. Danielson repair of Ebstein's malformation and other intracardiac repair were performed on all patients, followed by bi-directional cavopulmonary anastomosis, on pump with heart beating. Results There was no death, and no severe heart dysfunction and refractory low cardiac output in all patients postoperatively. No reoperation for residual or recurrent tricuspid incompetence was required in all patients. At follow-up of 13 (72.2%) patients ranging from 1 to 53 months, 12 patients were in New York Heart Association ((NYHA)) class I, 1 in class II. Four were with 1 grade, 2 with 2 grades tricuspid regurgitation. The patency of bi-directional cavopulmonary anastomoses was verified by echocardiography. Conclusion Ventricular unloading added to intracardiac repair appears to be effective to improve left and right ventricular function and tricuspid valve performance in Ebstein's anomaly with moderate or massive tricuspid dysfunction and physiological right ventricular outlet tract obstruction.

A Modified Technique in Surgical Correction of Ebstein Anomaly / 대한흉부외과학회지

There are various surgical techniques in repairing Ebstein anomaly, but residual tricuspid regurgitation and compromized right heart function may ensue in some cases. We report our clinical experience of Ebstein anomaly and atrial flutter in a 19-year-old male patient who underwent simple modified tricuspid annuloplasty, bi-directional cavopulmonary shunt and cryoablation of cavotricuspid isthmus.

A Modified Technique in Surgical Correction of Ebstein Anomaly / 대한흉부외과학회지

There are various surgical techniques in repairing Ebstein anomaly, but residual tricuspid regurgitation and compromized right heart function may ensue in some cases. We report our clinical experience of Ebstein anomaly and atrial flutter in a 19-year-old male patient who underwent simple modified tricuspid annuloplasty, bi-directional cavopulmonary shunt and cryoablation of cavotricuspid isthmus.