Mechanical support for bridge to transplant in an infant with post-cardiotomy end-stage heart failure and complete heart block: report of a case.

The patient was diagnosed with perimembranous ventricular septal defect (VSD). She underwent VSD closure and muscle bundle resection across right ventricular outflow tract at the age of 3 months. Since then, she had suffered from severe heart failure and complete heart block. Permanent pacemaker generator was implanted in the left hypochondrium. She was depended on continuous catecholamine administration, so transferred to our hospital for further management. On arrival, her body weight was 5686 g (- 2.7 SD). She underwent Excor pediatric left ventricular assist device implantation at the age of 9 months. Because the position of the left ventricular assist device cannula interfered with the pacemaker, herein, the pacemaker pocket was newly created in the left thoracic cavity. An 1 mm in thickness of expanded polytetrafluoroethylene sheet was trimmed and sutured under the anterolateral wall of left thoracic cavity as a pacemaker pocket. Bipolar ventricular lead was sutured on left ventricular apex and basal wall to face each other, mimicking cardiac regeneration therapy. Even though she unfortunately required right diaphragmatic plication for iatrogenic phrenic nerve palsy, her respiratory function was well maintained; therefore, secondary right heart failure was not observed. Her cardiopulmonary function was quite stable until post-operative day 275 when the patient was transferred to another hospital for heart transplantation.

Completely detached mechanical aortic valve prosthesis stuck to the aortic arch in a patient with Behçet's disease.

BACKGROUND: Although detachment of the implanted valve prosthesis was a well-known complication in patients with Behçet's disease, complete detachment of an aortic bileaflet valve prosthesis has never been reported. CASE PRESENTATION: An 18-year-old boy with Behçet's disease (HLA-A26 positive) who had previously undergone aortic valve replacement with an 18-mm ATS-Advanced Performance (ATS-AP) valve (ATS Medical, Inc., Minneapolis, MN) at the age of 12 years, presented sudden-onset general fatigue and was emergently transferred to the regional hospital. Chest X-ray showed displacement of the implanted mechanical valve. An echocardiogram revealed mobile valve prosthesis and severe aortic regurgitation. Just before leaving for our hospital for surgical treatment, a completely detached valve prosthesis was floating in the ascending aorta. On arrival, the valve prosthesis was stuck to the transverse arch. Emergent removal of the previous mechanical valve from the aortic arch and redo aortic valve replacement with a 24-mm ATS-AP valve were performed under total circulatory arrest. Infectious endocarditis was denied by histopathological examination. The patient was back to the intensive care unit with extracorporeal membrane oxygenation support, which was successfully decannulated 5 days later. CONCLUSIONS: This was the first report of a patient with Behçet's disease who encountered a complete detachment of implanted aortic valve prosthesis. The patient could be rescued by emergent surgery.

A rare case of pediatric primary cardiac tumor in a patient with Down syndrome.

Although hematological malignancies are a known complication of Down syndrome, few reports have described cases involving solid tumors. We describe the case of a 3-year-old Down syndrome girl with a primary solid cardiac tumor. Outpatient echocardiography after intracardiac repair of a ventricular septal defect at 6 months of age revealed a highly mobile pedunculated mass (8 × 9 mm) on the free wall of the right atrium. Due to potential incarceration of the mass in the tricuspid orifice, it was excised under extracorporeal circulation and cardiac arrest. Macroscopically, the tumor closely resembled a papillary fibroelastoma, although histopathological tests were inconclusive.

A Permanent Epicardial Pacemaker Lead That Penetrated the Esophageal Wall 26 years After Implantation.

We treated a patient in whom a permanent epicardial pacemaker lead penetrated the esophageal wall 26 years after the index pacemaker implantation. A 28-year-old man with loss of appetite and weight loss underwent upper gastrointestinal endoscopy and was found to have a foreign body protruding into the esophagus. Computed tomography revealed an epicardial lead penetrating the esophageal wall. He had undergone pacemaker implantation with permanent epicardial leads when he was 2 years old. The lead was surgically removed.

Evaluation of 60 cases of systemic-pulmonary shunt with cardiopulmonary bypass.

OBJECTIVE: In recent years, the median sternotomy approach with cardiopulmonary bypass has been increasingly chosen when systemic-pulmonary shunt surgery is performed as initial palliation for congenital heart diseases with decreased pulmonary blood flow to secure a stable surgical field and to maintain a stable circulation and oxygen supply. Since 2007, this strategy has been applied in our institute. This time, we examine the advantage and disadvantage of this procedure by evaluating the intraoperative and postoperative courses. METHODS: The study investigated 60 cases that underwent systemic-pulmonary shunt surgery under cardiopulmonary bypass at our facility after August 2007. Original diagnosis, age and body weight at surgery, shunt procedure, concomitant procedure, and surgical times were evaluated. The postoperative course of each case and the results of subsequent surgeries were also examined. RESULTS: No death or severe complication occurred during surgery or in the perioperative period. The age at surgery was 4 days-12 years (median 5.1 months), and the body weight was 2.3-28.1 (median 4.7) kg. Surgical procedures were as follows: right modified Blalock-Taussig shunt (mBTS): 35, left mBTS: 11, and central shunt: 14. In addition, the following simultaneous surgeries were conducted: pulmonary artery plasty: 11, unifocalization: 5, main pulmonary artery ligation: 2, interatrial communication enlargement: 4, and total anomalous pulmonary venous drainage repair: 2. CONCLUSION: The outcomes at our facility have validated the safety of systemic-pulmonary shunt surgeries under cardiopulmonary bypass, and even the disadvantages of concern were believed to be within acceptable limits. Further innovation and examination are important in pursuit of even less invasive surgeries.

Coronary re-implantation after completion of neo-aortic reconstruction in arterial switch operation: accurate intraoperative assessment for the optimal re-implantation site.

The arterial switch operation (ASO) has evolved into the treatment of the choice for most forms of transposition of the great arteries (TGA). Despite advancement in the technical aspects of the procedure, certain anatomical variations of the coronary arteries are still considered as surgical risks. We have recently employed a novel technique for coronary artery reconstruction in ASO to achieve further improvement of coronary transfer in cases with complex coronary anatomy. The technical key of the procedure is that reconstruction of the coronary arteries is preceded by neo-aortic anastomosis. After neo-aortic reconstruction is accomplished, the neo-aorta is temporarily distended with removal of the cross-clamp. The distended neo-aorta informs us its postsurgical geometry, which facilitates accurate assessment for the optimal site of coronary button transfer. The technique was feasible in 13 of 15 children who were consecutively treated by our group between 2003 a nd 2008. All patients recovered uneventfully and no coronary perfusion issue has occurred during the follow-up period. However, the complex anatomy of the coronary arteries in two children was not amenable to this technique. One with double loops (1RL; 2Cx) accompanied by side-by-side relationship of the great arteries underwent the open trapdoor technique, while the other with intramural coronary artery underwent the Imai method, that is one of procedure in which the coronary arteries are left in situ. The coronary re-implantation after neo-aortic reconstruction is promising to minimize postsurgical coronary ischemia and suitable for most ASO cases. However, various modifications of coronary transfer are required in a few variations of the coronary anatomy and we have to pursue further technical refinement of coronary artery transfer in ASO.

Surgical management of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is a rare but life-threatening disorder, particularly in neonates and infants. The stenotic lesions are commonly composed of complete rings of cartilage varying in length, location, and severity. A definitive diagnosis of CTS may be delayed because of the rarity of this disorder and thus its unfamiliarity among physicians, its variable onset time, its various clinical symptoms, and the diversity of associated clinical conditions due to the cardiovascular disorders that may accompany it. More than half of the patients who manifest clinical symptoms during early infancy show a long-segment stenosis. Long-segment CTS is problematic and challenging to manage. Over the last two decades several surgical techniques for long-segment CTS have been devised and developed, but no definitively advantageous surgical procedure has been established because of insufficient experience and the lack of large-scale studies. Although rib cartilage tracheoplasty and pericardial patch tracheoplasty have provided limited early to midterm success for infants with long-segment CTS, these procedures are associated with early and late complications, including granulation tissue formation, patch collapse, and restenosis necessitating reintervention. By contrast, slide tracheoplasty has given rise to better surgical outcomes. This procedure enables reconstruction of the stenotic trachea using native tracheal walls with preserved blood supply. The trachea is foreshortened by only one-half of the original stenosis, resulting in reduced tension on the anastomosis. Consequently, the technique has several advantages, including less formation of granulation tissue, satisfactory subsequent growth, and infrequent reoperation for restenosis. Slide tracheoplasty is currently recognized as the preferred technique for long-segment CTS.

Experience with nifekalant hydrochloride in a patient with ischemic cardiomyopathy and severe ventricular dysfunction after dor operation.

A 52-year-old male with ischemic cardiomyopathy and severe ventricular dysfunction underwent coronary artery bypass grafting and left ventricular reconstruction (Dor operation). The patient developed acute onset of incessant ventricular tachycardia in the early postoperative period that was refractory to therapy with class I antiarrhythmic agents, and multiple attempts at electrical cardioversion were required. A combination of intravenous nifekalant hydrochloride and enteral amiodarone was elected as treatment for this recurrent incessant ventricular tachycardia. Nifekalant hydrochloride was administered as a loading dose (0.3 mg/kg/5 min), followed by an intravenous infusion (0.4 mg/kg/hr). Several days after initiating therapy, the patient no longer experienced episodes of ventricular tachycardia, and there was no compromise in hemodynamics. We conclude that nifekalant hydrochloride is a useful agent for suppression of ventricular tachycardia in patients with severe left ventricular dysfunction, especially during the early postoperative period.

Efficacy of endoventricular circular patch plasty: will this procedure improve the prognosis of patients with ischemic cardiomyopathy?

OBJECTIVES: We report hemodynamic and clinical results of our series of endoventricular circular patch plasty (Dor operation) and consider some advantages of this procedure for patients with ischemic cardiomyopathy. METHODS: Between 1996 and 2001, 23 consecutive patients with left ventricular aneurysm and/ or ischemic cardiomyopathy after myocardial infarction who underwent Dor operation were included in this study. Hemodynamic and clinical results of Dor operation were analyzed periodically. Patients were divided into two groups according to the extent of asynergy, more than 60% or not, i.e., an ischemic cardiomyopathy group (ICM group) or a simple left ventricular aneurysm group (sLVA group). RESULTS: Hospital mortality was 4.4%. Postoperative New York Heart Association functional class was improved in all survivors to class I or II. Postoperative ejection fraction (EF) increased and postoperative left ventricular (LV) volume decreased in all survivors. In both groups, early and 1 year postoperative EF increased significantly. Additionally, end-diastolic and end-systolic volumes decreased significantly in the early postoperative period. Postoperative LV volume had re-enlarged in the cases in which preoperative left ventricular end-systolic volume index was more than 90 mL/m2, though left ventricular ejection fraction was maintained or rather improved at 1 year postoperatively. The survival rates after 3 years of the operation in the sLVA and ICM groups were 85.7% and 81.3%. CONCLUSION: Though patients with ischemic cardiomyopathy with severe LV dysfunction may benefit the most from Dor operation, postoperative LV re-dilatation may deteriorate late mortality. At operation, whether optimal LV size and shape can be reconstructed and the timing of operation are the important issues because they affect prognoses.

Changes in left ventricular volume and predictors of cardiac events after endoventricular circular patch plasty.

OBJECTIVE: The aim of this study was to identify predictors of cardiac events after endoventricular circular patch plasty (Dor operation) by analyzing our experience with Dor operation. METHODS: Thirty patients with left ventricular aneurysm and/or ischemic cardiomyopathy who underwent Dor operation were included in this study. Hemodynamic and clinical results were analyzed, and the predictors of cardiac events were examined. RESULTS: Hospital mortality was 3.3%. Postoperative clinical status and left ventricular (LV) function in all survivors significantly improved. The survival rates at 1, 3, and 5 years after operation were 93%, 89% and 89%. The corresponding cardiac event-free rates were 75%, 67% and 49%. Pre- and postoperative LV function and volume did not differ significantly between patients with or without cardiac events. However, the proportion of reduced end-diastolic volume index (EDVI) (preoperative EDVI-postoperative EDVI) to preoperative EDVI was significantly higher in patients with cardiac events than in cardiac event-free patients. Postoperative LV volume re-increased in the cases with cardiac events during follow-up. Cox regression analysis confirmed that preoperative clinical premature ventricular contraction and end-systolic volume index (ESVI), postoperative EDVI, ESVI, and ejection fraction were independent predictors of late cardiac events. There was a significant positive correlation between preoperative ESVI and postoperative EDVI. CONCLUSION: Though LV function significantly improved after Dor operation, LV reconstruction with excessive reduction can cause restarting LV remodeling and increasing mortality and morbidity. Therefore, LV reconstruction of appropriate sizes and shapes, considering the function of residual myocardium, has a significant effect on prognosis. It is highly reasonable to expect that preoperative ESVI can predict the optimal size of reconstructed left ventricle.

The limitation of staged repair in the surgical management of congenital complex heart anomalies with aortic arch obstruction.

OBJECTIVE: Severe aortic arch obstruction including an interrupted aortic arch in congenital complex heart anomalies remains a challenge in surgical management. METHODS: Treatment and outcomes in 75 consecutive patients who underwent an aortic arch repair as the first step of the staged repair protocol between 1975 and 2000 were reviewed. Their ages at repair ranged from 1 day to 8.5 months. RESULTS: Cross-sectional postoperative follow-up data were available in all the patients. The follow-up period ranged from 0 to 27.6 years (mean: 7.3 +/- 7.3 years). There were 20 postoperative hospital deaths (27%) and 7 late deaths. The Kaplan-Meier estimate of survival was 81.3% +/- 4.5% at 1 month, 68.0% +/- 5.4% at 1 year, 65.0% +/- 5.5% at 5 years, 63.1% +/- 5.7% at 10 years, 63.1% +/- 5.7% at 20 years. By Cox regression analysis, body weight of 2.5 kg or less is the only independent determinant of postoperative mortality (p = 0.04, multivariable odds ratio: 2.50, [95% confidence interval: 1.02-6.1]). The aortic arch morphology, the primary cardiac lesion, or date of operation did not reach a statistically significant level to show correlation with mortality. Reintervention to reconstruct the aortic arch was performed at 9 occasions in 8 of the 55 patients who survived the primary operation (14.5%). The Kaplan-Meier estimate of the reintervention-free rate was 91.3% +/- 4.2% at 5 years, 85.5% +/- 5.6% at 10 years, 75.6% +/- 8.2% at 20 years. Using multivariable Cox regression analysis, interrupted aortic arch (versus aortic coarctation) was the only independent predictor of a shorter time to reintervention (p = 0.001, multivariable odds ratio: 16.1, [95% confidence interval: 3.2-80.2]). CONCLUSIONS: The staged repair protocol was associated with significant limitations in patient survival and with the development of recurrent aortic arch obstruction. Thus, a primary repair protocol may serve as an alternate approach, especially in patients with low weight or with an interrupted aortic arch.