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Preoperative and postoperative management in patients with pulmonary hypertension associated with congenital heart disease is improving by understanding of pathophysiology of pulmonary arterial hypertension (PAH) and several types of pulmonary vasodilators. While the irreversible PAH in untreated lesions has become rare in Japan, persisting/progressive PAH even after timely surgery remain challenging. To optimize preoperative and postoperative management in patients with PAH associated with congenital heart disease, adequate indication and treatment algorithms should be constructed.

Bochdalek hernia is the most common type of congenital diaphragmatic hernia( CDH). Patients with CDH often develop cardiopulmonary dysfunction secondary to pulmonary hypoplasia and pulmonary hypertension, which necessitates emergency surgery. Advanced cardiopulmonary management is essential in such cases;therefore, patients should be treated at a well-facility that is familiar with the treatment of CDH. Respiratory management using gentle ventilation and control of pulmonary hypertension using nitric oxide are necessary for preoperative stabilization of patients. Intraoperatively, the hernia should be cautiously reduced and contents should be returned into the abdominal cavity. Surgery is performed to secure the circumference of the rim and ensure firm closure of the defect. Thoracoscopic surgery may be considered for late-onset cases with a stable general condition.

A right-sided classical Blalock-Taussig shunt was created in a 3-year-old male infant with hypoplastic left ventricle, common atrioventricular valve, transposition of the great arteries, and pulmonary stenosis. He was diagnosed with near Eisenmenger pulmonary hypertension at the age of 6 years. Surgery to achieve right heart bypass circulation was not conducted. At 33 years of age, he experienced hemoptysis. Pulmonary artery dissection, a life-threatening condition, was detected upon computed tomography performed when the patient was 35 years of age. After three weeks, surgery with cardiopulmonary bypass requiring pulmonary artery replacement using composite prosthetic conduits, closure of the pulmonary valve, and banding of the previously created Blalock-Taussig shunt was performed. Six years after the surgery, the patient's physical activity is maintained. Although intracardiac repair or definitive palliation with cardiopulmonary bypass is contraindicated in such patients, life-saving surgery with cardiopulmonary bypass that does not worsen pulmonary hypertension can be performed.

A large population of patients with congenital heart disease with untreated systemic to pulmonary shunts( left to right shunts) will develop pulmonary arterial hypertension( PAH). There are 2 different statuses of an increase in pulmonary arterial pressure. One is high resistance due to high pulmonary blood flow (high flow with high resistance), another one is low pulmonary flow due to high resistance (low flow with high resistance). Chronic large left-to-right shunt induced severe pulmonary vascular disease and pulmonary hypertension. This was then subsequence of low pulmonary blood flow with high pulmonary vascular resistance. We have to avoid this situation and have to do intervention within the pulmonary vascular reactivity has been left. For this reason, preoperative treatment for avoidance of high flow, appropriate timing of interventions and postoperative various managements are important factors as aiming of low pulmonary resistance in this group. Recent advances in PAH-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes "treatment" with PAH-specific drugs initially and then "repair" by closure of the cardiac defect (i.e. "treat and repair") was devised, and has been attempted, in patients with PAH associated with a cardiac defect.

Although many patients with a ventricular septa defect undergo primary intra-cardiac repair surgery, some patients require 2-stage repair with pulmonary artery banding. Patients with 21-trisomy, who exhibit fragility of the pulmonary vasculature and respiratory systems, may be candidates for this approach. Herein, we report the case of a 21-trisomy patient with ventricular septal defect associated with pulmonary hypertension. She underwent pulmonary artery banding at 4 months of age, and a subsequent radical operation at 27 months. Serial pathological findings between pre- and post-pulmonary artery banding revealed that changes of pulmonary vasculature and respiratory systems were reversible. Her Heath-Edwards grade changed from 0 from II and index of pulmonary vasculature disease changed from 1.1 to 1.0. In conclusion, pulmonary artery banding in a case with pulmonary hypertension prevented the lung from becoming irreversible pathological changes.

We report a case of a 60-year-old man with postoperative congestive heart failure( CHF) successfully treated with tolvaptan. The patient was diagnosed with pulmonary hypertension due to mitral stenosis and regurgitation combined with tricuspid regurgitation. He underwent mitral and tricuspid valvuloplasty. His postoperative course was uneventful until CHF symptoms secondary to volume overload appeared on the 4th day. Congestion with pulmonary hypertension was treated with 0.042 μg/kg/min of intravenous human atrial natriuretic peptide (hANP). His condition improved, and on the 11th postoperative day, he was weaned off hANP;oral administration of 40 mg per day of furosemide was initiated. However, 2 days after discontinuation of intravenous hANP, CHF recurred and serum sodium decreased to 128 mEq/l. Oral tolvaptan 7.5 mg per day was added to the furosemide, and CHF and hyponatremia subsequently improved. In this case, oral tolvaptan was effective for the treatment of refractory CHF with pulmonary hypertension after cardiac surgery.

A large population of patients with congenital heart disease with relevant systemic to pulmonary shunts( left to right shunts) will develop pulmonary arterial hypertension if left untreated. There are 2 different statuses of an increase in pulmonary arterial pressure. One is high resistance due to high pulmonary blood flow( high flow with high resistance), another one is low pulmonary flow due to high resistance( low flow with high resistance). Chronic large left-to-right shunt induced severe pulmonary vascular disease and pulmonary hypertension. This was then subsequence of low pulmonary blood flow with high pulmonary vascular resistance. We have to avoid this situation and have to do intervention while the pulmonary vascular reactivity has been left. Therefore accurate diagnosis, preoperative treatment for avoidance of high flow, appropriate timing of interventions and post operative various managements are important factors as aiming of low pulmonary resistance in this group. It is important to be familiar with.

We report a case with the rare association of craniofacial anomalies, cerebellar vermis hypoplasia (Dandy-Walker variant) and congenital heart disease referred to as 3C (cranio-cerebello-cardiac) syndrome. A male infant was born at 40 weeks' gestation. The birth weight was 2,896 g. At birth he had macrocephaly, widely open metopic suture, ocular hypertelorism, cleft palate, apparently low-set ears, cerebellar vermis hypoplasia and ventricular septal defect (VSD) with pulmonary hypertension. Chromosomes were normal and we diagnosed 3C syndrome. At 8 months of age we performed a cardiac catheterization. The pulmonary artery pressure was 70/26 (48) mmHg and pulmonary vascular resistance (RpU) was 8.5 U/m2. But under the oxygenation RpU decreased to 1.5 U/m2. At 10 months of age we performed VSD patch closure. After operation his pulmonary artery pressure was 39/13 (25) mmHg under the oxygen therapy. He was on good course and discharged at 58 post operative day.

A 4-month-old female with ventricular septal defect (VSD) and severe pulmonary hypertension (PH) underwent a patch closure for VSD. She could not be weaned from cardiopulmonary bypass (CPB) after the intracardiac repair due to PH crisis. Nitric oxide inhalation therapy during partial CPB enabled her to be weaned from CPB. This therapy could be gradually taped off and quit 7 days after the operation. Nitric oxide inhaled therapy is considered to be an excellent treatment for PH crisis during CPB in congenital cardiac surgery. The causes of PH crisis were also discussed in reference to the date of endothelin-1 (ET-1) measured during the operation.

A 24-year-old pregnant female was admitted to our hospital because of congestive heart failure. When she was 3 month old, she underwent closure of patent ductus arteriosus. Echocardiography, cardiac catheterization and selective angiocardiography showed a left ventricular-right atrial shunt. At the operation, we found a left ventricular right-atrial communication (group I) and repaired the defect directly. The patient was discharged and returned to her social life.

We report about successful case of human atrial natriuretic peptide (HANP) infusion therapy for early infants who developed congestive heart failure after surgical repair of congenital heart diseases at Ehime Prefectural Central Hospital between January, 1998 through January, 2000. Age at operation ranged from 0 day to 38 days (mean 20 days), and body weight ranged from 1.6 kg to 3.5 kg (2.8 kg as mean). Following HANP infusion at incremental doses of 0.05 to 0.24 microgram/kg/min (0.16 microgram/kg/min as mean), urine output and hemodynamics dramatically improved. Urine output increased from 0.75 ml/kg/hr to 2.79 ml/kg/hr (p = 0.0001) and good urinary output was maintained (3.06 ml/kg/hr) even after discontinuation of the infusion. During the infusion, the heart rate decreased from 157 bpm to 150 bpm and the systemic systolic blood pressure increased from 68 mmHg to 78 mmHg. Central venous pressure decreased from 9.3 mmHg to 7.9 mmHg. There were no adverse effects by HANP infusion. These are successful cases of HANP infusion therapy as the initial treatment of post-operative pulmonary hypertension in this age group. Human atrial natriuretic peptide infusion therapy can be used safely and might be useful in some early infants with CHF, although more investigation is needed.

A 47-year-old man was receiving anticoagulant therapy after coronary artery bypass grafting and mitral valve plasty. A chest roentgenogram disclosed alveolar infiltrates throughout both lung fields 6 months postoperatively. Lung biopsy specimens showed the presence of hemosiderin-laden macrophages in the alveolar spaces, establishing a diagnosis of diffuse alveolar hemorrhage. Anticoagulant therapy may have caused the hemorrhage, because the patient had no immunologic disorder or renal disease. The shadows completely disappeared after steroid therapy and discontinuation of anticoagulant therapy. Diffuse alveolar hemorrhage should be considered, when extensive infiltrates develop on chest roentgenogram in patients receiving anticoagulant therapy after open heart surgery. Lung biopsy is essential, when a bronchoalveolar lavage fails to diagnose the disease.

A 10-month-old girl was diagnosed with atrial septal defect and pulmonary sequestration and scimitar syndrome. Cardiac catheterization revealed severe pulmonary hypertension, with systemic pulmonary artery pressure 97 mmHg, pulmonary resistance 10.3 U, and Qp/Qs 0.45. Systemic pulmonary artery pressure was decreased from 108 to 75 mmHg by oxygenation. We performed coil embolization of the systemic artery and repair of the partial anomalous pulmonary venous return, division and reimplantation of the anomalous vein into the right atrium, and a patch was inserted to redirect flow to the left atrium. The severe pulmonary hypertension was improved and echocardiography showed systemic pulmonary artery pressure of 45 mmHg. The postoperative hemodynamic and respiratory condition was satisfactory.

A 10 month-old infant with Down syndrome having ventricular septal defect and pulmonary hypertension performed cardiac catheterization, which resulted in a slight increase in the pulmonary vascular resistance from 10.8 to 11.3 unit.m2. Lung biopsy findings showed at most an early grade 2 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.1, both of which indicated operability for total correction. He underwent surgical correction and the pulmonary arterial pressure significantly decrease. Although he suffered chylothorax in 5th postoperative day, he did not developed pulmonary hypertension crisis.

A 61-year-old woman with mitral valve regurgitation and pulmonary hypertension, was referred to us for surgical repair. Preoperative left ventriculography showed Sellers IV mitral valve regurgitation and high pulmonary arterial pressure: 103/31 (57) mmHg. The mitral valve was replaced with a phi 27 mm SJM mechanical valve. The postoperative residual pulmonary hypertension was successfully treated with nitric oxide gas, decreased significantly pulmonary arterial pressure, and the postoperative course was uneventful. Based on our experience, we think that nitric oxide gas may prove effective for residual pulmonary hypertension after a cardiac operation.

A 70-year-old man was admitted with syncope attack on exertion. Ventricular septal defect with pulmonary hypertension and left atrial myxoma were confirmed by ultrasonography and cardiac catheterization. Preoperative Pp/Ps was 0.95 and pulmonary vascular resistance was 16 units. Pulmonary vascular resistance decreased to 9.6 units by the administration of Isoproterenol and decreased to 8.5 units with PGE1. Patch closure of VSD and excision of left atrial myxoma were performed simultaneously. The patient recovered completely, although he suffered from pneumonia and jaundice due to liver congestion postoperatively. Cardiac catheterization before discharge revealed Pp/Ps 0.38 and PVR 10.1 units.

A 57-year-old woman was admitted to our hospital for the treatment of mitral regurgitation and giant bulla with severe pulmonary hypertension. A dobutamine-induced test performed preoperatively resulted in a decrease of the systolic pulmonary artery pressure by 30 mmHg. Subsequently, mitral valve replacement and bullectomy were performed concomitantly. The patient recovered from heart failure, and the pulmonary artery pressure clearly decreased during the perioperative period. This case report serves to demonstrate the effectiveness of performing a one-staged operation for mitral regurgitation and giant bulla with severe pulmonary hypertension.

A 51-year-old male was diagnosed as having an ostium secundum atrial septal defect with severe pulmonary hypertension. Although pulmonary artery pressure was as high as 96/32 and pulmonary vascular resistance was 14.2 U.m2, he underwent corrective surgery, because pulmonary to systemic blood flow ratio was 2.0. After he regained consciousness in ICU, pulmonary hypertensive crises recurrently occurred and the hemodynamics became quite unstable. Administration of conventional drugs proved to be ineffective. Since inhalation of low dose (3-4 ppm) nitric oxide was started, however, his condition was markedly improved and he recovered uneventfully thereafter. Two years later, pulmonary artery pressure was reduced to 52/28 and pulmonary vascular resistance was 9.9 U.m2. Although good outcome can not be expected in all surgically treated cases, we should refrain from deciding easily that surgery is contraindicated, because no criteria of surgical indication for atrial septal defect with pulmonary hypertension appears to be perfect.

We performed surgery for a partial endocardial cushion defect (ECD) in a 67-year-old female patient with pulmonary hypertension. The patient had grade 2 tricuspid regurgitation and pulmonary hypertension (Pp/Ps = 0.58, Rp = 8.4 Um2). The operative procedure involved patch closure of the ostium primum and tricuspid annuloplasty with a Carpentier ring. The postoperative course was uneventful and the pulmonary hypertension improved (Pp/Ps = 0.33). Surgery for partial ECD should be recommended even for elderly patients.

A case of congenital mitral stenosis, patent ductus arteriosus, pulmonary hypertension was reported. At one year of age, the patient underwent surgical division of PDA because of persistent left heart failure. She went well after the operation. At seven year of age, she was readmitted to our hospital for easy fatigability. The cardiac catheterization revealed remarkably elevated pulmonary arterial pressure and pulmonary capillary wedged pressure. She underwent a surgical intervention for the mitral stenosis at eight year of age. At operation, the mitral valve exhibited the characteristics of type IIc according to Carpentier's classification: thickened and dysplastic leaflet, extremely short chordae tendanae fused with papillary muscles, obliteration of interchordal space and hypertrophic two papillary muscles. We replaced the valve with 23 mm Carbomedicus prosthetic valve because it seemed to be difficult to repair the native valve satisfactorily without residual stenosis or insufficiency. Her postoperative course was uneventful and the pulmonary arterial pressure and pulmonary capillary wedged pressure decreased remarkably one year after the operation.

A 40-year-old man was diagnosed as having PDA with severe PH by cardiac catheterization. We decided that surgery was indicated on the basis of 100% oxygen inhalation test. After 100% oxygen inhalation, left to right shunt changed from 37.8% to 61.5%, Qp/Qs changed from 1.43 to 2.60, and PVR changed from 1,199 dyne/sec/cm5 to 237 dyne/sec/cm5. We decided to perform surgery. Under extracorporeal circulation, PDA was safely closed by triple ligation and was directly closed after pulmonary arteriotomy. The postoperative course was uneventful. Severe PH was greatly improved, pulmonary artery pressure recovering to the normal range, by administering PGE1, prostacyclin (PGI2), and nitroglycerin.

Severe (110/50 mmHg) pulmonary hypertension (PH) secondary to mitral valve prolapse is rare. We reported herein severe PH secondary to mitral valve prolapse in a 65-year-old female. The patient underwent mitral valve replacement and tricuspid annuloplasty. The pulmonary artery pressure after the operation decreased to 69/20 mmHg. Prostagrandin E1 was effective for residual PH compared with nicardipine hydrochloride.

We encountered a 40-year-old male patient diagnosed to have an atrial septal defect combined with pulmonary hypertension and left main coronary artery stenosis of 50% caused by a dilated pulmonary artery. Preoperative Trazoline tolerance test revealed an increase in pulmonary artery pressure from 77/22(40) to 85/24(42), and a decrease in pulmonary vascular resistance from 4.2 U.m2 to 3.6 U.m2. Moreover, his treadmill exercise test was positive. We performed only a patch closure of the atrial septal defect. The postoperative catheterization examination showed normal pulmonary artery pressure, mild mitral valve regurgitation, and improvement of the left main coronary artery stenosis from 50% to 41%. After 6 months, his treadmill exercise test was negative. We believe that it is important to estimate preoperatively for the possible reversibility of pulmonary hypertension in a patient with an atrial septal defect combined with a left main coronary artery stenosis caused by a dilated pulmonary artery.

A one year old boy with the distal type of aortopulmonary window was successfully repaired by the transaortic approach and discharged two weeks after operation. Exposure of the defect was enhanced by using deep hypothermia with circulatory arrest so that aortic cannula and cross-clamp could be removed. 2 months before the operation, he needed the respiratory support during a month because of respiratory syncytial viral infection, cardiac failure and equivalent pulmonary hypertension. Postoperative cardiac catheterization after 4 months revealed that PA pressure decreased to 25/5 mmHg without any medication. Aortogram showed the left single coronary artery.

The surgical correction was performed successfully in a 3-year-old girl with tetralogy of Fallot associated with total anomalous pulmonary venous drainage (type Ia). The preoperative cardiac catheterization and angiography showed high systolic pulmonary artery pressure (58 mmHg) and small left ventricular volume (54% of normal). The early postoperative course was stormy because of the unstable circulatory state. During the early postoperative period, we estimated the volume of left ventricle by 2 D echocardiography. A significant increase of the volume was observed on the 8th postoperative day, when her circulatory state became stable. The cardiac catheterization before discharge revealed Pp/Ps of 0.38 and normal LV volume. We conclude that primary repair should be done for this combined cardiac anomaly, even if small left ventricle and pulmonary hypertension is present.

Between 1989 and 1992, atrial septal defect secundum (ASD) were closed in 8 patients aged 51 to 77 years with the use of cardiopulmonary bypass. The notable characteristics in our cases were; 1) All but one had slight or moderate left to right shunt ratios. Although pulmonary hypertension was present, the obstructive pulmonary vascular change had not progressed so far. 2) Atrial fibrillation was present in 6 patients (75%) and some other cardiac lesions (atrioventricular insufficiency, ischemic heart disease, intracardiac anomaly) coexisted with ASD in a high frequency. And surgical procedures other than ASD closure were required in 5 patients. 3) We must take care to prevent cardiac complications (i.e., arrhythmia) as well as extracardiac malfunctions in postoperative care, because aged patients had some organic dysfunctions of respiratory, renal and hepatic systems. 4) Post-operative courses were satisfactory and there was no operative mortality. All patients obtained improvements in their quality of life. Consequently, we concluded that surgical closure is recommended even for aged patients with ASD, except for Eisenmenger's syndrome.

This paper describes a new operative method for patent ductus arteriosus using left heart bypass. A 57-year-old woman with patent ductus arteriosus associated with severe pulmonary hypertension was successfully operated upon. Because of aneurysmal dilatation and calcification of the aortic side of the ductus, PDA was divided under left heart bypass with centrifugal pump. Postoperative course was uneventful, and is now well.

Twelve patients with ventricular septal defect with Down syndrome (Down group) underwent cardiac surgery. As they had pulmonary hypertension (Pp/Ps > or = 0.5), we chose twenty patients who had pulmonary hypertension (Pp/Ps > or = 0.5) with ventricular septal defect without Down syndrome as control group. After cardiac surgery, Pp/Ps in Down group decreased significantly from 0.81 +/- 0.14 to 0.48 +/- 0.25 (p < 0.01). Cardiac index (l/min/m2) between 24 hours after operation was 3.3-3.8 in Down and 4.1-4.8 in control group (NS). Oxygenation index (mmHg) between 24 hours after operation was 211-266 in Down and 230-314 in control group (NS). Eleven out of 12 patients in Down group had good operative result. One patient was complicated with post-operative pulmonary hypertensive crisis. Low output syndrome and death were not encountered in Down group. We were satisfied with operative results and encourage surgical treatment for ventricular septal defect with Down syndrome.

From 1968 to 1987, 45 consecutive patients with tetralogy of Fallot and pulmonary atresia underwent corrective surgery. In the former 10 years, there were 8 operative deaths (40%) in the 20 patients and no late death. In the recent 10 years, there were 4 operative deaths and 4 late deaths in the 25 patients. In the 17 patients associated with large aorto pulmonary collateral artery (LAPCA) which was ligated in the most patients, there were 5 operative deaths (29%) and 3 late deaths. Nine patients (53%) survived long-term. There were 7 operative deaths (25%), one late death and 20 patients (71%) being long-term survivors in the 28 patients without LAPCA. Pulmonary hemodynamics were investigated in 10 patients without LAPCA and 11 with LAPCA after corrective surgery. Pulmonary arterial mean pressure (PAm) ranged from 9-24 (17 +/- 6) mmHg in patients without LAPCA and 15-92 (37 +/- 23) mmHg in those with LAPCA. Pulmonary arteriography showed arborization abnormality in 7 of the 11 patients with LAPCA. PAm inversely correlated well with number of pulmonary artery segments connected to the central pulmonary arteries. Postoperative pulmonary hypertension was found in patients with less than 16 bronchopulmonary segments connected to the central pulmonary arteries, and two of them died of pulmonary hypertension late after surgery. These results suggested that unifocalization to connect the central pulmonary artery more than 15 bronchopulmonary segments may be an important strategy for correction of this anomaly with LAPCA.

A 31-year-old woman had a left atrial myxoma associated with severe pulmonary hypertension and respiratory failure because of incarceration of the tumor to the mitral valve. Emergency surgery was performed, but the patient could not be weaned from cardiopulmonary bypass because of right ventricular failure. A Biomedicus centrifugal pump was used as a right ventricular assist pump. The result was a successful termination of cardiopulmonary bypass. With the support of an intra-aortic balloon pump, the right ventricular assist pump was removed 38 hours after the operation, and the intra-aortic balloon pump was terminated 2 days later. The centrifugal pump is very useful at the majority of centers where the pneumatically activated bi-valved ventricular assist device is unavailable. The Biomedicus centrifugal pump can be easily applied for treatment of perioperative right ventricular failure and is very useful for short-term use without systemic anticoagulants.

The effectiveness of various methods of myocardial protection were evaluated retrospectively in 59 infants less than 12 months of age who underwent open heart surgery for ventricular septal defect with severe pulmonary hypertension. Intermittent aortic clamping and electrically induced ventricular fibrillation (EF) were employed in 13 infants (Group I), and potassium induced cold cardioplegia and topical cardiac cooling (TC) were used in 14 infants (Group II). Six infants in Group II had additional EF after declamping of the aorta (Group II-A) but the rest of Group II infants did not have any EF (Group II-B). Profound hypothermia and circulatory arrest were utilized in 17 infants (Group III). Cold blood cardioplegia with TC were used in 15 infants (Group IV). Moderate hypothermia were used during cardiopulmonary bypass in Group I, II and IV. The operative mortality for Group I was 15% and was 0% for Group II, III and IV. The incidence of spontaneous resumption of cardiac beat following declamping of the aorta were 33.3, 0, 100, 94.1, 93.3% for Group I, II-A, II-B, III and IV respectively. The urinary output obtained in the postoperative 72 hours was significantly lower in Group I than in Group II, III and IV (Group I less than II less than IV less than III).(ABSTRACT TRUNCATED AT 250 WORDS)

A 22-year-old man with patent ductus arteriosus with severe pulmonary hypertension was operated upon successfully. The operation, trans-aortic direct closure of ductus, was done using temporary long external bypass. Postoperative course was uneventful, and the patient is now well 6 years and 8 months after operation. Postoperative cardiac catheterization revealed decreased pressure of pulmonary artery from 116 to 44 mmHg. The details of the operative procedure and postoperative course were described and a brief review of literatures was also done.