Patients with double-inlet left ventricle usually have a small ascending aorta. In the Norwood procedure, which involves a staged operation, a neoaorta is constructed with a homograft, and the pulmonary artery plays a role in the systemic circulation. Dilatation or aneurysmal changes can occur over time due to the exposure of the neoaorta to systemic pressure, which may induce adverse effects on adjacent structures. We report a rare case of surgical repair for neoaortic root dilataiton with aortic regurgitation, compressing the left pulmohary artery, in a patient who underwent the Norwood procedure.
Allografts ; Aneurysm ; Aorta ; Aortic Valve Insufficiency ; Arteries ; Dilatation ; Fontan Procedure ; Heart Ventricles ; Humans ; Norwood Procedures ; Pulmonary Artery

BACKGROUND: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. MATERIAL AND METHOD: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was 17.3+/-10.8 days (range: 9~36 days) at the 1st staged operation, 8.9+/-7.1 months (4.6~23.3 months) at the 2nd staged operation (the Bidirectional Glenn procedure) and 32.4+/-9.8 months at the final staged operation (the Fontan procedure). During the 2nd staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. RESULT: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was 19.6+/-14.9 months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). CONCLUSION: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.
Aspirin ; Echocardiography ; Follow-Up Studies ; Fontan Procedure ; Hemodynamics ; Humans ; Hypoplastic Left Heart Syndrome ; Medical Records ; Norwood Procedures ; Polytetrafluoroethylene ; Retrospective Studies ; Tricuspid Valve ; Tricuspid Valve Insufficiency ; Ventricular Function

BACKGROUND: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. MATERIAL AND METHOD: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, 11.7+/-13.2 days) and weight at operation ranged from 2.2 to 4.8 kg (mean, 3.17+/-0.52 kg). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean 26.0+/-22.8 months). RESULT: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. CONCLUSION: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.
Aneurysm ; Cardiovascular Abnormalities ; Deglutition Disorders ; Follow-Up Studies ; Fontan Procedure ; Hospital Mortality ; Humans ; Hypoplastic Left Heart Syndrome ; Male ; Norwood Procedures ; Pulmonary Artery ; Risk Factors ; Subclavian Artery ; Survival Rate

No abstract available.
Norwood Procedures* ; Palliative Care* ; Postoperative Care* ; Pulmonary Artery*

A patch design using bovine pericardial patch for aortic enlargement in the Norwood procedure has been introduced to avoid problems related to homograft availability. We report 2 successful cases of Norwood procedure with home-made bovine pericardial patch. The first case was a 23-day-old (2.2 kg) patient with multi-level left ventricular outflow tract obstruction with ductal-dependent systemic circulation. The other case was a 9-day-old (3 kg) patient with hypoplastic left heart syndrome. This technique was relatively easy to perform, reproducible and quite effective like homograft patch.
Allografts ; Humans ; Hypoplastic Left Heart Syndrome ; Norwood Procedures*

Modified Norwood procedure with maintaining cardiac beat was done in a 30-day-old neonate. Procedure was done with regional perfusion of innominate and coronary artery. Postoperative course was uneventful. Second-stage operation (bi-directional cavopulmonary shunt) was done 4 months later. The diameter of ascending aorta was more than 5 mm, Norwood procedure can be done in beating hearts.
Aorta ; Coronary Vessels ; Heart Defects, Congenital ; Heart* ; Humans ; Infant, Newborn ; Norwood Procedures* ; Perfusion

Mediastinitis and sternal wound infection frequently occurred after corrective surgery for complex heart anomalies. Most of the patients are neonate or infant and they have showed low oxygen tension. For that poor condition, application of invasive treatment of mediastinitis is not appropriate and recovery takes a long time. We successfully treated a mediastinitis after Norwood operation using vacuum assisted closure. So we introduce the technique and the modification of neonatal vacuum assisted closure.
Heart ; Humans ; Infant ; Infant, Newborn ; Mediastinitis* ; Negative-Pressure Wound Therapy* ; Norwood Procedures* ; Oxygen ; Vacuum* ; Wound Infection

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome(n=7), mitral stenosis with small VSD and subaortic stenosis(n=1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis(n=1), interrupted aortic arch with ventricular septal defect and subaortic stenosis(n=1), tricuspid atresia with transposition of the great arteries(n=1), and complex double-inlet left ventricle(n=2). All patients without hypoplastic left heart syndrome were associated with hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months(mean 60.6+/-71.6 days, median 39 days). The operative mortality(<30 days) was 46%(6 patients). Late mortality was 15%(2 patients). All operative deaths occured during the first 24 hours after the operation as a result of cardiopulmonary bypass weaning failure(5 patients) and sudden hemodynamic instability postoperatively(1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals(39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and late death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.
Aorta ; Aorta, Thoracic ; Aortic Coarctation ; Cardiopulmonary Bypass ; Fontan Procedure ; Heart ; Heart Bypass, Right ; Heart Defects, Congenital* ; Heart Diseases ; Heart Septal Defects, Ventricular ; Hemodynamics ; Humans ; Hypoplastic Left Heart Syndrome ; Infant* ; Mitral Valve Stenosis ; Mortality ; Norwood Procedures* ; Pneumonia, Aspiration ; Survival Rate ; Tricuspid Atresia ; Weaning

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.
Aorta ; Aorta, Thoracic* ; Esophagus ; Heart Defects, Congenital ; Humans ; Hypoplastic Left Heart Syndrome* ; Norwood Procedures* ; Pulmonary Artery ; Subclavian Artery ; Trachea

A 12-day-old female baby underwent a Norwood procedure for hypoplastic left heart syndrome. The left superior vena cava (LSVC), which was found incidentally during the operation, was divided to facilitate surgical exposure. After the operation, she developed signs of low cardiac output and died 7 hours afterward. Autopsy findings showed that the coronary sinus was atretic at the orifice without unroofing into both atria, rendering the LSVC the sole route of coronary sinus drainage. In patients with incidentally-found LSVC during surgery, special care should be taken to leave the LSVC intact because the LSVC may be the exclusive drainage vein of the coronary venous system.
Autopsy ; Cardiac Output, Low ; Coronary Sinus ; Drainage ; Female ; Humans ; Hypoplastic Left Heart Syndrome ; Norwood Procedures ; Veins ; Vena Cava, Superior