Echocardiographic Evaluation of Postoperative Coaptation Geometry of Left AV Valve in Complete Atrioventricular Septal Defect.

Background: We sought to determine the difference in geometric parameters in the left atrioventricular valve (LAVV) postoperative complete atrioventricular septal defect (CAVSD) compared to the normal heart, and the correlation between geometric and functional parameters for detecting the mechanism of LAVV regurgitation (LAVVR) in CAVSD. Methods: LAVV geometric parameters based on complete and acceptable quality echocardiograms of 18 patients with repaired CAVSD compared with 17 normal controls. LAVVR severity was also quantified by indexed vena contracta (I-VC) (mm) and % jet area/left atrium area (% Jet/LA), and the correlation with LAVV parameters in the CAVSD group was investigated. Results: In the CAVSD group, the posterior closing angle (Pc) was nearly the same as the anterior closing angle (Ac), yet in the normal heart, the Pc angle was double the Ac angle. The anterior opening angle (Ao) and posterior-to-anterior leaflet diameter ratio (a/p) in the CAVSD group was also significantly smaller. The CAVSD group also had a shorter indexed coaptation length (I-CL) and indexed tenting height (I-TH). Displacement length (ΔD) differed completely between the CAVSD and Normal groups, and also showed a strong positive correlation to the functional parameters of LAVVR (% Jet/LA: r = .70, P = .02; I-VC: r = .60, P = .02). Conclusions: The parameters in this study were applicable to CAVSD AV valve coaptation characteristics. We introduced 2 novel measures that may provide important insights into the differences in geometry and performance of the LAVV in repaired CAVSD as compared to normal hearts.

[A New-born Case of Total Conus Defect and Single Coronary Artery with Situs Inversus Totalis Suspected Kartagener Syndrome].

We report a new-born case of total conus defect type ventricular septal defect (VSD) and single coronary artery with situs inversus totalis, suspected Kartagener syndrome clinically. After the birth, as the patient had suffered from respiratory distress due to high pulmonary blood flow through the large defect, surgery was planned at age of 14-days after birth. Under median sternotomy and cardiac arrest, patch closure of VSD was performed as ordinary fashion. In spite of the situs inversus totalis and single coronary artery arose from right coronary sinus, operator could have completed all of surgical procedure at the right side of patient as usual. No remarkable respiratory complication was seen postoperatively and she was discharged from hospital 18th day in a good condition.

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.

Hybrid Palliation for Interrupted Aortic Arch With Small Aortic Valve.

OBJECTIVES: Open heart surgery for interrupted aortic arch in the neonatal period is still a high-risk procedure related in part to patient factors such as low birth weight, other morphologic anomalies, and, especially, small aortic valve size. Recently, we performed hybrid palliation with bilateral pulmonary artery banding and ductal stenting as the first-stage palliation for such cases. In this study, the outcomes of this procedure were examined. METHODS: Six cases of interrupted aortic arch with a small aortic valve underwent the hybrid procedure in the neonatal period in our institute from 2010 to 2015 (mean age: 6.8 days, mean body weight: 3.2 kg, mean z score of the aortic valve annulus: -8.3). Their postoperative clinical courses and results of the second-stage surgery were evaluated. RESULTS: No mortality or severe morbidity was seen in association with initial hybrid palliation. Five of six patients were discharged from the hospital; the one exception had a significant urinary tract anomaly. None needed an additional catheter intervention or surgical procedure postoperatively. All surviving patients underwent second-stage surgery; three had biventricular repair by the conventional method or Damus-Kaye-Stansel anastomosis with the Rastelli procedure and the other three proceeded toward staged Fontan reconstruction. Growth of the aortic valve was seen in four patients, and increased indexed left ventricle volume was recognized in one after the palliation. CONCLUSION: Hybrid palliation could be useful not only to avoid high-risk neonatal surgery but also to allow for eventual selection of the second-stage surgery based on the observations of potential interval development of left ventricular structures.

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.

Inflammatory response to hyperoxemic and normoxemic cardiopulmonary bypass in acyanotic pediatric patients.

BACKGROUND: Hyperoxemic management during cardiopulmonary bypass (CPB) is still common, and there is no consensus about physiologic oxygen tension strategy (normoxemic management) during pediatric CPB. In this study, we compared the postoperative conditions and measures of inflammatory response among patients with acyanotic congenital heart disease subjected to either hyperoxemic or normoxemic management strategy during CPB. METHODS: We studied 22 patients with a ventricular septal defect and pulmonary artery hypertension. The patients were divided into two groups. Group I (n=9) received normoxemic management (PaO2=100-150 mm Hg) and group II (n=13) received hyperoxemic management (PaO2=200-300 mm Hg) during CPB. There was no difference between groups with regard to age, body weight, duration of CPB, and aorta clamping time or preoperative pulmonary hypertension (pulmonary pressure/systemic pressure [Pp/Ps]). In each group, the blood samples to measure the cytokine levels were collected before and after the CPB. RESULTS: Although we observed no statistically significant differences in postoperative intubation time, alveolar-arterial oxygen difference, creatine kinase MB level, and pulmonary hypertension (Pp/Ps) between group I (10.7±13.4 hours, 197±132 mm Hg, 148±58.6 IU/L, 42.8%±22.1%, respectively) and group II (27.8±36.5 hours, 227±150 mm Hg, 151±72.6 IU/L, 50.4%±16.0%, respectively), levels of median interleukin 6 and tumor necrosis factor α were lower in group I (129.8 and 17.0 pg/mL, respectively) than that in group II (487.8 and 22.5 pg/mL, respectively). CONCLUSION: During the CPB in acyanotic pediatric patients, normoxemic management can minimize the systemic inflammatory response syndrome associated with CPB. We can apply this physiologic oxygen tension strategy to surgical advantage during heart surgeries in acyanotic pediatric patients.

Influence of bidirectional cavopulmonary anastomosis and concomitant valve repair on atrioventricular valve annulus and function.

BACKGROUND: The relationship between atrioventricular valve regurgitation (AVVR) and valve annulus after bidirectional cavopulmonary anastomosis (BCPA) and adequate indications for valve repair are unclear. METHODS: We evaluated the size of the valve annulus and the grade of AVVR before and immediately after BCPA, and at the most recent follow-up before the Fontan operation in 37 patients with a functional single ventricle. RESULTS: Nine patients underwent concomitant valve surgery. The mean z value of the valve annulus was significantly lower postoperatively than preoperatively in the 28 patients who were not treated by valve surgery (0.45 vs 1.51, p=0.01). However, mean regurgitation scores did not significantly change after BCPA (1.60 vs 1.78, p=0.08). The most recent assessment showed that the mean z value increased compared with that immediately after BCPA (1.36 vs 0.45, p=0.005). This increase was significant in the patients with moderate regurgitation. The mean z value of the valve annulus of the patients treated by concomitant valvuloplasty was significantly lower postoperatively than preoperatively (-0.25 vs 3.9, p=0.0001) and remained low at the latest evaluation. Mean regurgitation scores also significantly decreased after BCPA (2.25 vs 3.37, p=0.007). CONCLUSIONS: Unloading the systemic ventricle by BCPA leads to a decrease in the relative size of the atrioventricular valve. However, this decrease does not improve the degree of AVVR in the absence of concomitant valve repair. Concomitant valve repair is justified in patients with moderate or worse AVVR and an abnormal valve structure.

Cardiopulmonary bypass through a left thoracotomy using venous drainage from the innominate vein in congenital heart surgery.

Cardiopulmonary bypass (CPB) through a left lateral thoracotomy is a useful approach for some congenital heart procedures, although vascular access for the arterial and venous cannulation can be challenging in the selective patients. Six patients underwent successful extracorporeal circulation through a left lateral thoracotomy using the innominate vein for venous drainage. No operative deaths or major complications occurred. Venous drainage solely from the innominate vein was adequate to establish partial bypass without the need for pericardiotomy. Total bypass was established with combined venous drainage from the innominate vein and the main pulmonary artery. Exposure of the systemic atrioventricular valve was excellent through a left thoracotomy. Venous drainage from the innominate vein without using atrial drainage can safely be used for extracorporeal circulation through a left lateral thoracotomy without compromising the procedure and it is a useful approach to congenital heart surgery in selected patients.

Modified Norwood procedure with a handmade down-sizing valved right ventricle-to-pulmonary artery conduit.

First-stage palliation of hypoplastic left heart syndrome has been revolutionized by the recent introduction of a right ventricle-to-pulmonary artery (RV-PA) conduit as an alternative to a systemic-to-pulmonary shunt. However, most conduits are unvalved, and the use of valved xenografts was abandoned during the early era of this operation. We performed a successful modified Norwood operation in a 2-month-old infant with aortic atresia and ventricular and atrial septal defects using a hand-made down-sizing valved graft as an RV-PA conduit. The postoperative course was uneventful with well-balanced pulmonary and arterial perfusion. We believe that minimization of the regurgitant volume from an unvalved prosthetic conduit by utilizing this modification might be of benefit in this particular group of patients.

Scimitar syndrome in an infant with right lung hypoplasia, ventricular septal defect, and severe pulmonary hypertension.

A 5-month-old boy was presented for surgical repair of scimitar syndrome associated with right lung hyperplasia, severe pulmonary hypertension, ventricular septal defect (VSD), and atrial septal defect. The calculated shunt fraction (Qp/Qs) was 3.1:1.0, pulmonary vascular resistance was 4.6, and the perfusion lung scan showed a marked decrease (11%) in right pulmonary blood flow. Surgical repair was performed through the right fourth intercostal space with the patient supine. The anomalous vein was divided and interposed with a short azygos vein graft, followed by closure of the VSD. Finally, the interposed azygos vein was anastomosed to the left atrium. Although pulmonary artery pressure was normalized at the 2-year follow-up, cardiac catheterization 6 months after the operation demonstrated right pulmonary vein obstruction.

Pediatric left atrial myxoma: surgical excision and mitral valve plasty.

This report describes the case of a 12-year-old girl with a giant left atrial myxoma who presented with severe mitral regurgitation symptoms. Echocardiography demonstrated a 69 x 30 mm solid mass in the left atrium (LA), occupying almost the entire mitral orifice. After successful surgical excision of the tumor, concomitant with mitral valve plasty, there was no clinical or echocardiographic recurrence at 12-month follow-up.

Double switch operation for congenitally corrected transposition of the great arteries after two-staged pulmonary artery banding.

We describe a case of congenitally corrected transposition of the great arteries (cc-TGA) successfully performed by the double switch operation after two-staged pulmonary artery banding (PAB). An eleven-year old boy diagnosed with cc-TGA underwent the first PAB at that age, followed by the second PAB one year later. Because of severe ventricular dysfunction and arrhythmia of the anatomic left ventricle, the intension of one-stage PAB was abandoned. Cardiac catheterization data from after the adequate second PAB provided the surgical indication for the anatomical correction and double switch operation (Senning+Jatene procedure) and this was successfully performed at age 14. Although cardioversion was required to treat supraventricular tachycardia in the early period after surgery, the patient was discharged from hospital and remains in good clinical condition at the last follow-up at 5 years with normal sinus rhythm and good biventricular function.