Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.
Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. Material and Method: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3-120 days) and 3.8$\pm$0.7kg (range, 2.92-5.3kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1 LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. Result: Overall postoperative hospital mortality was 23.1 % (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3$\pm$30.7 months. Pulmonary valvar stenosis (>30mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. Conclusion: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients Without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.
Kim, Jeong-Young;Im, Hyo-Bin;Son, Sang-Hee;Jeong, So-Young;Sung, Min-Jung;Seo, Son-Sang
Neonatal Medicine
/
v.16
no.1
/
pp.76-80
/
2009
A male baby with intrauterine growth retardation had a short neck, small hands and feet, hypospadia, both grade I hydronephrosis, type II atrial septal defect, and moderate valvular pulmonary stenosis. The routine chromosome and banding analyses revealed a 46,XY,rec(8)del(8)(p21)dup(8) (q24.1)inv(8)(p21q24.1)pat chromosome constitution. His mother has normal chromosomes, but the father had 46,XY,inv(8)(p21q24.n Also his uncle had an inv(8) chromosome constitution. We used lymphocytes and examined 40 mitotic cells. All mitotic cells showed deletion of 8p21-->pter and duplication of 8q24.1 -->qter. Because Sp21 involves secretion of macrophage and lymphocyte against cancer cells, long-term follow-up for cancer will be needed.
A 4-year-old female Fox Terrier dog (weighting 8 kg) with history of severe abdominal distention and anorexia was presented to Veterinary Teaching Hospital, Gyeongsang National University. In physical examination, grade 4/6 systolic murmur heard at the left heart base. Electrocardiography showed right ventricular enlargement (right axis deviation and deep S wave) and right atrial enlargement (P pulmonale). Diagnostic imaging studies revealed hypertrophy of right ventricle, enlargement of right atrium and tricuspid regurgitation and turbulence in the pulmonary artery in right parastenal short axis view. Based on the diagnostic findings, the dog was diagnosed as a case of valvular pulmonic stenosis.
A two-month-old female Yorkshire terrier was referred to the Veterinary Teaching Hospital, College of Veterinary Medicine, Kyungpook national University. The patient was presented with a history of dyspnea, cough, exercise intolerance and abdominal distension, but she was appetence. In physical examination the puppy was coughed on slight tracheal compression. Rectal temperature, pulse and respiratory rate were normal, and grade 3/6 systolic murmur heard at the left heart base. The murmur was crescendo-decrescendo. Electrocardiography showed sinus arrhythmia, right-ventricular hypertrophy pattern, and right axis deviation. Thoracic radiography revealed cardiomegaly, bulging of the main pulmonary artery, and enlarged left side heart. Abdominal radiography revealed abdkominal distention. Echocardiography showed hypertrophy of right ventricle and turbulence in the pulmonary artery in parasternal oblique view. Subvalvular pulmonic stenosis was diagnosis based upon the clinical signs, physical examination, electrocardiography, radiography and echocardiography. We treated the patient with furosemide, enalapril and $\beta$-blocker. After the clinical signs of cough, abdominal distension and dyspnea were disappeared, she was on just $\beta$-blocker for prevention of occurrence of congestive heart failure. Now she was recovered her health, and she is not on any medication.
Background: Reoperation is usually required for a right ventricle to pulmonary artery conduit obstruction caused by valve degeneration, conduit peel formation or somatic growth of the patient. An autologous tissue reconstruction (peel operation), where a prosthetic roof is placed over the fibrotic tissue bed of the explanted conduit, has been used to manage conduit obstructions at our institute since May 2002. Herein, the early and midterm results are evaluated. Material and Method: Between May 2002 and July 2006, 9 patients underwent obstructed extracardiac conduit replacement with an autologous tissue reconstruction, at a mean of 5.1 years after a Rastelli operation. The mean age at reoperation was $7.5{\pm}2.4$ years, ranging from 2.9 to 10.1 years. The diagnoses included 6 pulmonary atresia with VSD, 2 truncus arteriosus and 1 transposition of the great arteries. The preoperative mean systolic gradient was $88.3{\pm}22.2mmHg$, ranging from 58 to 125 mmHg. The explanted conduits were all Polystan valved pulmonary conduit (Polystan, Denmark). A bioprosthetic valve was inserted in 8 patients, and a monocusp ventricular outflow patch (MVOP) was used in 1 patient. The anterior wall was constructed with a Gore-Tex patch (n=7), MVOP (n=1) and bovine pericardium (n=1). Pulmonary artery angioplasty was required in 5 patients and anterior aortopexy in 2. The mean cardiopulmonary bypass time . was 154 minutes, ranging from 133 to 181 minutes; an aortic crossclamp was not performed in all patients. The mean follow-up duration was 20 months, ranging from 1 to 51 months. All patients were evaluated for their right ventricular outflow pathway using a 3-D CT scan. Resuit: There was no operative mortality or late death. The mean pressure gradient, assessed by echocardiography through the right ventricular outflow tract, was 20.4 mmHg, ranging from 0 to 29.6 mmMg, at discharge and 26 mmHg, ranging from 13 to 36 mmHg, at the latest follow-up (n=7, follow-up duration >1 year). There were no pseudoaneurysms, strictures or thrombotic occlusions. Conclusion: A peel operation was concluded to be a safe and effective re-operative option for an obstructed extracardiac conduit following a Rastelli operation.
Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.
Kim, Seong-Jun;Kang, Min-Hee;Kim, Su-Chan;Choi, Young-Chul;Kim, Seung-Gon;Lee, Chang-Min;Jung, Da-Min;Park, Hee-Myung
Journal of Veterinary Clinics
/
v.31
no.4
/
pp.325-328
/
2014
A 8-month-old female Maltese dog was presented with a history of heart murmur. In physical examination, grade 4/6 systolic murmur heard at the left heart base. Electrocardiography showed sinus arrhythmia, right axis deviation, deep S wave and splintered QRS complex. Thoracic radiography revealed enlarged right side heart and bulging of the main pulmonary artery. Echocardiography showed mild hypertrophy of right ventricle, a supravalvular stenosis, marked post-stenotic dilation of the main pulmonary artery and a moderately increased pulmonary arterial velocity through the stenotic area (4.4 m/s, pressure gradient of 78.7 mmHg). The dog was diagnosed with supravalvular pulmonic stenosis based on the diagnostic imaging findings. Medical management using ${\beta}1$-blocker and ACE inhibitor was started in this dog and this is first case report described diagnostic characteristic features of supravalvular pulmonic stenosis in korea.
In recent years, the use of allograft conduits in repair of congenital cardiac disease is widely accepted. However, the supply of homograft. is currently limiting their increased clinical application, especially small cryopreserved homografts for use in neonates and inf'ants. We used a technique to surgically reduce the size of the more readily available large-diameter allografts, making them suitable for right ventricular outflow tract reconstruction in small infants and children. From December 1994 to March 1996, a total of 11 patients ranging in age from 10 months to 6 years (mean age, 27.3 months) and ranging in weight from 5.6 to 18.5 kg (mean 11.5 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique (pulmo ary atresia with ventricular septal defect, 9 cases ; tetralogy of Falloff, 2 cases). The diameter after downsizing ranged from 14 to 19 mm with a mean of 16.8 mm. There was one operative death due to rupture of the infected homograft. Evaluation of these patients between 2 and 15 months (mean 6.9 months) after homograft implantation reveals excellent clinical and echocardiographic results. There were no significant homograft insufficiency and RVOT obstructions. Although a longer follow-up is certainly required to evaluate the long term fate of the surgically modified bicuspid homografts, we believe that this technique may represent a valuable therapeutic alternative, at least in the short term, to the use of synthetic grafts when an appropriately sized homograft is not available.
Background: Anatomic and functional abnormalities of the systemic atrioventricular (AV) valve are common in single ventricle. pathologies and continue to be associated with poor early and late outcomes in surgically palliated single. ventricle patients. We aggressively performed valvuloplasty for atrioventricular valve regurgitation (AVVR) during the course toward a Fontan operation. Material and Method: Between January 1995 and December 2004, 209 patients underwent a Fontan operation in our institution. We retrospectively evaluated the prevalence of AVVR and the influence of AV valve repair on outcome, and we analyzed the progression of AVVR after the Fontan operation for 168 patients where echocardiographic follow up results for more than 6 months after the Fontan operation were available. During the course toward a Fontan operation, 25 patients underwent 30 procedures for AVVR. These procedures. were. carried out during placement of a bidirectional cavopulmonary shunt (BCPS) for nine patients, between the time of placement of a BCPS and the Fontan operation for four patients, and during the Fontan operation for 17 patients. Five patients underwent procedures for AVVR twice. Result: The late mortality rate after the Fontan operation was 4.2% (n=7), with a median follow-up duration of 52 months (range, $6{\sim}123$ months). Seven patients (4%) had unfavorable outcomes such as significant (moderate or severe) AVVR in six patients, and significant AV valve stenosis in one patient was determined at the last follow up after the Fontan operation. Among the seven patients, four patients underwent AV valve repair after the Fontan operation, and one patient underwent subsequent AV valve replacement. Progression to AVVR of equal to or greater than grade 2 was noted in 30 patients (18%) at the last follow up after the Fontan operation, including 12 patients that underwent previous AV valve procedures. Initial grading of AVVR, a previous AV valve operation, and specific AV valve morphology such as a common AV valve or mitral atresia were significant risk factors for the progression of AVVR after the Fontan operation. Conclusion: In our surgical series, a small percentage of patients showed unfavorable outcomes. related to AVVR during the course toward a Fontan operation. However, a closer follow-up is required to evaluate the progression of the AVVR after a Fontan operation, especially for patients showing poor AV valve function at the first presentation and specific AV valve morphology.
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