[Staged surgical palliations for hypoplastic left heart syndrome with poor preoperative conditions].

A 2.8 kg-female newborn diagnosed as hypoplastic left heart syndrome was transferred to our hospital after resuscitation against systemic circulatory failure due to closure of the ductus arteriosus. Her systemic circulation was stabilized after alprostadil (lipo-PGE1) and inotorpic agent infusion along with mechanical ventilation support. On admission, signs of a pulmonary high-flow and low systemic perfusion were evident. Impaired coagulation aspect was gradually noted. Because a surgical palliation with cardiopulmonary bypass was considered to be at high-risk, a bilateral pulmonary artery banding was indicated. Through a median sternotomy, the right and left pulmonary arteries were individually banded. The patient was continued on the same ventilation strategy. Cardiac, hepatic and renal dysfunctions were improved over next few days and the patient weaned from ventilatory support. She underwent combined Norwood stage I and II repair at 4 months of age with weight of 4.8 kg. Postoperative course has been uneventful and the patient is now followed up in preparation for Fontan operation.

Mean xenograft survival of 14.6 days in a small group of hDAF-transgenic pig hearts transplanted orthotopically into baboons.

INTRODUCTION: In a discordant orthotopic xenotransplantation model (pig-to-baboon) donor pigs expressing human decay accelerating factor (hDAF) as a regulator of complement activity were used to prevent hyperacute xenograft rejection (HXR). We investigated a modified immunosuppressive therapy consisting of ERL080 (Novartis Pharma AG, Base, Switzerland), cyclosporin A (Neoral), steroids, and a cyclophosphamide (CyP) induction protocol with several reduced doses to prevent acute vascular rejection (AVR). METHODS: Donor hearts were harvested from hDAF-transgenic pigs (18.8 +/- 2.6 kg, Imutran Ltd., a Novartis Pharma AG Company). Four adult baboons (25.6 +/- 2.7 kg) with high titers of xenoreactive antibodies (XAb) served as recipients. Serological and hemodynamic parameters were measured. Finally, myocardial tissue was sampled for histological and immunohistochemical examinations. RESULTS: In the first baboon, an acute graft failure occurred after 1 hour due to preservation injury. The second succumbed after 11.1 day due to an acute renal failure. The third died after 13.1 days of an ileus. The fourth baboon had continuously excellent cardiac function (mean echocardiographic ejection fraction, 69.2%), but succumbed on day 20 due to anemia. Corrected mean xenograft survival (excluding the first baboon because of a technical failure) was 14.6 +/- 2.6 days. XAb decreased after day 3 to constantly low levels (<1:64 titer) after CyP induction. White blood cell count decreased from 10.3 +/- 0.8 to 0.9 +/- 0.3 G/L after day 3. Macroscopically and histologically no typical signs of HXR or severe AVR could be detected. CONCLUSIONS: These results confirm that hDAF transgen blocks HXR in this life-supporting model. AVR was prevented by using a modified quadruple immunosuppressive drug combination (Neoral, ERL080, steroids, and several small single doses of CyP). An optimum "fine-tuning" of immunosuppression is required to achieve the best risk-benefit ratio.

Combination of hDAF-transgenic pig hearts and immunoadsorption in heterotopic xenotransplantation of immunosuppressed baboons.

INTRODUCTION: Hyperacute xenograft rejection (HXR) and acute vascular rejection (AVR) after xenotransplantation are triggered by xenoreactive antibodies (XAb) and an activated complement cascade. In a heterotopic (abdominal) xenotransplantation model we combined immunoadsorption (IA, Ig-Therasorb column) and a quadruple immunosuppressive drug therapy in recipient baboons with donor pig hearts transgenic for human decay accelerating factor (hDAF). METHODS: According to XAb titers between 6 and 14 cycles of IA were performed preoperatively in 4 recipient baboons (18.6 +/- 2.5 kg). Hearts of hDAF-transgenic donor pigs (6.1 +/- 1.1 kg, Imutran Ltd., a Novartis Pharma AG Company, Basel, Switzerland) were heterotopically transplanted using the abdominal technique in baboons. Immunosuppression consisted of cyclophosphamide (CyP) induction therapy, ERL080 (Novartis Pharma AG), cyclosporin A (CyA, Neoral), and steroids. Blood levels of mycophenolate, CyA, immunoglobulins (Ig), anti-pig-antibodies, complement factors, and cardiac enzymes were determined. Abdominal electrocardiography (ECG), echocardiography, and palpation were used for monitoring of the pig hearts. Myocardial tissue specimens were examined using immunohistochemistry, light microscope (LM), and electron microscope (EM). RESULTS: Ten cycles of IA alone removed 78% of XAb and accordingly IgM, IgG, IgA, complement C3, and C4. None of the xenografts was hyperacutely rejected, but xenograft failure occurred after 5.0 +/- 1.3 days (range, 2.4-8.0 days) because of an AVR associated with a rapid XAb increase within 24 hours. White blood cell count (10.3 +/- 2.2 G/L) showed a maximum of 13.1 +/- 2.1 (day 1) and constant levels (1.4 +/- 0.3-2.1 +/- 1.3 G/L) between day 3 and 6. Histology (LM/EM) showed massive hemorrhage, necrosis, and vascular thrombi as signs of AVR. CONCLUSION: Although HXR was prevented by using IA and hDAF-transgenic donor hearts, AVR was not avoided due to insufficient immunosuppressive regimen used and a missed postoperative IA treatment as a result of an inefficient control of XAb production.

Primary cardiac angiosarcoma with superior vena caval syndrome: review of surgical resection and interventional management of venous inflow obstruction.

Primary cardiac angiosarcoma occurs rarely, and surgical resection is often required to relieve symptoms. A 66-year-old man whose presenting symptoms were palpitations and general fatigue is presented. Echocardiography revealed a large tumour occupying most of the right atrium. When superior vena caval (SVC) syndrome developed, surgical resection of the tumour was attempted. The tumour was found to have invaded the inflow of SVC, left atrium, right ventricle and ascending aorta. The bulk of the tumour was resected, requiring reconstruction of the right atrium and caval inflows. Histopathological diagnosis of the tumour was primary angiosarcoma. In the postoperative period, symptoms of SVC syndrome recurred, which were relieved temporarily by balloon angioplasty. After one month, recurrence of symptoms was again managed by catheter dilation. On this occasion a metallic stent was deployed, which prevented further symptoms of SVC syndrome during the eight months before the patient died with generalized metastases.

[A surgical case of supravalvular aortic stenosis with severe hypoplastic ascending aorta (diffuse type) in Williams-Beuren syndrome].

We report a six-year-old boy who underwent ascending aortic reconstruction for supravalvular aortic stenosis of diffuse type associated with Williams-Beuren syndrome. The diagnosis was first made at the age of six months. Because of progressive left ventricular hypertrophy, cardiac catheterization was performed at the age of five years and showed left ventricular pressure of 200 mmHg, the ascending aortic pressure of 202 mmHg, the descending aortic pressure of 115 mmHg, and left ventricular end-diastolic volume of 33.5 ml (90% of normal). Whole ascending aorta except sinus Valsalva was severely hypoplastic, so called diffuse type of supravalvular aortic stenosis. The ascending aorta was only 6.3 mm in diameter, whereas the diameter of the aortic annulus was 20.6 mm. The ascending aortic reconstruction of Doty's type was performed from the aortic root to the distal aortic arch with a equine pericardium reinforced by Dacron velour using selective perfusion to the right bracheocepharic artery, the left common carotid artery and the right femoral artery to avoid circulatory arrest. Postoperative course was uneventful and post operative catheterization revealed left ventricular pressure of 128 mmHg, the ascending aortic pressure of 126 mmHg and the descending aortic pressure of 90 mmHg. Mild residual pressure gradient was probably due to hypoplastic descending aorta. In conclusion, patch aortic reconstruction for ascending aorta under selective cerebral perfusion for a six-year old boy can be performed without postoperative neurological complication.

[Color M-mode Doppler analysis of left ventricular inflow in pediatric patients].

This study evaluated the clinical usefulness of analyzing left ventricular (LV) filling by color M-mode Doppler echocardiography in pediatric patients. The LV-filling patterns of color M-mode Doppler echocardiography were obtained by LV inflow in the apical 4-chamber or long-axis view, and the time difference between the occurrence of peak velocity at the mitral tip and in the apical region (M-AP) was calculated. The peak velocity at each depth was determined by adequate selection of the Nyquist limit by shifting the zero point after freezing the color M-mode. LV volume and posterior wall motion velocity were obtained simultaneously. The catheter-derived data were compared with echo-derived data in 7 patients (mean age 12.0 years). The M-AP correlated positively with the time constant of LV relaxation (tau; r = 0.83, p < 0.05), pulmonary capillary wedge pressure (r = 0.83, p < 0.05), and negatively with peak diastolic posterior wall motion velocity (r = -0.78, p < 0.05). The M-AP was compared with other echo-derived data between Group N (35 children with normal cardiac function, mean age 4.3 years) and Group F (12 children with LV ejection fraction less than 40%, mean age 9.5 years). The M-AP was significantly longer in Group F (53.3 +/- 14.0 vs 116.5 +/- 30.5 msec; p < 0.001), but there was no significant difference in the E/A or deceleration time of E between the 2 groups. In Group N, the E/A correlated to LV end-diastolic volume and heart rate, but the M-AP showed no correlation. In Group F, the M-AP correlated with the percentages of normal LV end-diastolic volume (r = 0.76, p < 0.01) and LV ejection fraction (r = -0.58, p < 0.05). The M-AP was not influenced by LV size or heart rate and could easily differentiate normal heart from failing heart, and thus this is a useful parameter for evaluating diastolic function in pediatric patients.

[Surgical management of patent ductus arteriosus in low body weight infants].

Between January 1980 and December 1994, seventeen premature infants weighing less than 2500 g underwent surgical management for the isolated patent ductus arteriosus (PDA). Indometacine therapy for closure of PDA was not effective for all these patients. In terms of the body weight at birth, they were divided into two groups; Group I (G-I) consisted of ten patients with birth weight less than 1000 g and Group II (G-II) of seven patients weighing more than 1000 g. The age at operation was 22.1 +/- 15.4 days in the G-I and 14.3 +/- 11.4 days in the G-II. The ductus was ligated in all patients but one of the G-II, in whom it was divided. There were five (50%) hospital deaths in the G-I and none in the G-II. The causes of death in the G-I were related to necrotizing enterocolitis (NEC) in two and infant respiratory distress syndrome (IRDS), acute renal failure, and broncho-esophagial fistula in each. The age at operation tended to be older in nonsurvivors rather than in survivors in the G-I (28.0 +/- 16.8 days vs. 16.2 +/- 14.0 days, respectively, but the difference did not reach significance). Before surgery, all patients in the G-I required mechanical ventilator support and nine of them were associated with IRDS. In contrast, only two patients in the G-II needed mechanical ventilation preoperatively. The postoperative period of intubation was also significantly longer in the G-I than in the G-II (51.6 +/- 35.2 days vs. 2.2 +/- 1.5 days, respectively, p < 0.05). In conclusions, the surgery for PDA can be safely performed even in small premature babies weighing less than 1000 g. When medical therapy for PDA is not effective in the premature patients, the surgical management should be considered as early as possible before their conditions become worse due to such critical complications as NEC, IRDS, and renal failure.

[Total cavopulmonary connection for grown diminutive pulmonary artery after staged Blalock-Taussig shunt].

We report a two-year-old girl with asplenia, [A, L, L] DORV, pulmonary atresia, common AV valve, PDA, and TAPVC, who successfully underwent total cavo pulmonary connection (TCPC). Deep cyanosis was pointed out since birth. Cardiac catheterization performed on the sixth day after birth revealed a diminutive pulmonary artery tree of which PA index was 41 mm2/m2. Left modified Blalock-Taussig shunt was created at 27 days of age. The PA index increased to 282 mm2/mm2, but disparity in diameter between the left and the right pulmonary artery was yielded by PDA subsidence. Therefore additional contralateral B-T shunt was made at one year of age. Follow-up cardiac catheterization at 28 months of age showed well developed pulmonary artery; PA index of 460 mm2/m2, right pulmonary resistance (Rp) of 3.49 units, left Rp of 2.33 units, and estimated total Rp was 1.39. According to study, bidirectional Glenn procedure or TCPC was indicated. Considering necessity of urgent repair of common pulmonary vein obstruction, regurgitation of the common atrio-ventricular valve and pulmonary artery stenosis, TCPC was performed with concomitant repair of the associated lesions. Severe butterfly-figure stenosis of the central PA was augmented by anastomosing both the left SVC and the left-sided atrium. In conclusion, diminutive pulmonary artery could be adequately grown by phase-in Blalock-Taussig shunts. Pulmonary blood flow scintigraphy was thought to be useful for estimation of pulmonary resistance in such cases with different pulmonary resistance between right and left PA.

An automatic flow controller for a centrifugal blood pump.

To regulate the perfusion flow rate of a centrifugal blood pump, a microcomputer controller was developed. The computer monitored the flow rate of the pump with an electromagnetic flowmeter or an ultrasonic pulse Doppler flowmeter, rotational speed of the pump, aortic pressure, and the amount of blood in a reservoir. A discrete integral controller with a control interval of 1 s was adopted for the controller. For the safety of the control system, we added functions for detecting a clamp on the tubing, a dislocation of the flow sensor, or an inverse direction of the flow sensor. During a standby period, the computer calculated the rotational speed from aortic pressure to minimize the forward or the backward flow at the start of the pump perfusion. The automatic flow controller was used on 5 patients during cardiac operations and maintained the flow rate within +/-6% of the set point.

[A surgical case of total anterior papillary muscle rupture after acute myocardial infarction].

Mitral regurgitation (MR) due to rupture of the papillary muscle is one of the most serious complications of acute myocardial infarction (AMI) as well as ventricular septal perforation and ventricular free wall rupture. We experienced a case of complete rupture of the anterior papillary muscle. A 68-year-old man experienced an episode of dyspnea. Electrocardiographic findings were consistent with postero-lateral infarction. Massive MR is present on color Doppler imaging. He was transferred to our hospital for urgent operative indication because of papillary muscle rupture due to AMI. Six hours after the onset, the operation was performed with intra-aortic balloon pump in place. The anterior papillary muscle was ruptured completely in the mid portion. He underwent a mitral valve replacement with a SJM prosthetic valve. There is a few cases with successful urgent surgery for a complete rupture of anterior papillary muscle.

[Right atrial flap method for repair of partial anomalous pulmonary venous drainage into the high superior vena cava--case report].

We report an alternative method of repairing partial anomalous pulmonary venous return (PAPVR) wherein anomalous veins drain into the high portion of superior vena cava (SVC). A six year old girl, weighing 19 kg underwent cardiac catheterization for cardiomegaly. The study showed sinus venous defect and pulmonary veins anomalously drained from the right upper and middle lobe into the high SVC. Under cardiopulmonary bypass with aortic and bicaval direct cannulation, a 2-cm-wide pedicled flap made from the middle part of the right atrium (RA), was pulled into the SVC. The atrial flap divided the SVC from the ostium of the uppermost pulmonary vein (PV) to the ASD. This posterior tunnel drained anomalous PV blood into the left atrium. Postoperative echocardiogram and magnetic resonance imaging showed adequate space in the SVC and the PV channel. The patient maintained normal sinus rhythm after surgery. The advantage of this method is to minimize the risk of injuring the sinus node or sinus node artery, and to easily reconstruct both the SVC and PV channel without using prosthetic materials, even in patients with small RA.

[A case of modified Fontan operation with reconstruction of the nonconfluent pulmonary artery].

A three-year-old girl with complex cardiac anomalies and right isomerism successfully underwent a modified Fontan procedure and reconstruction of the nonconfluent pulmonary artery using autologous tissues. These cardiac anomalies included single right ventricle, atresia of the pulmonary trunk with nonconfluent pulmonary artery, atrial septal defect, common atrioventricular valve, bilateral PDAs, and bilateral SVCs. Preoperative cardiac catheterization showed elevated pulmonary artery pressure (mean pressure of 24 mmHg) and a small orifice of common pulmonary vein chamber. Pulmonary arteriographies showed balanced development of the branches. Indication for Fontan procedure was finally determined by measurement of the pulmonary artery and vein pressures and pulmonary vascular resistance before the cardiopulmonary bypass in the operation. Nonconfluent pulmonary artery was reconstructed by direct anastomosis of the right and left branches in the posterior aspect and enlarged with autologous pericardium patch. Intracardiac anomalies were repaired by enlargement of common pulmonary vein orifice (from 5 mm to 15 mm in diameter) and right atrial oblique partition. Furthermore, Fontan circulation was established by pulmonary artery angioplasty, direct anastomosis of left SVC to left pulmonary artery and direct anastomosis of right atrial appendage to pulmonary artery. Postoperative clinical course was uneventful, and cardiac catheterization showed mean right atrial pressure of 14 mmHg, cardiac index of 3.5 l/min/m2, and no pressure gradient at the site of reconstruction of the pulmonary artery.

[Surgical treatment of infective endocarditis : an analysis of the risk factor for operative mortality].

From January 1980, through December 1995, 60 patients underwent surgical treatment of infective endocarditis (IE) at Sakakibara Hospital. Of 60 patients, 40 showed active endocarditis and 20 healed endocarditis at the time of operation. The over-all hospital mortality was 10% (6/60). The complication of cerebral accident (embolism and bleeding) was related to a higher incidence of operative mortality. The reconstructions of destruction and disruption of ventricular-aortic or mitral-aortic continity in the presence of acute infection of the annular tissue were in need of radical and complex surgical techniques. It is recommended to follow up these patients thoughtfully and to perform reoperation or re-reoperation before a patient develops severe heart failure or multiple organ failure.

[Myocardial infarction caused by spontaneous coronary artery dissection associated with significant mitral regurgitation--a successful surgical case].

We experienced a case of 34-year-old male with old myocardial infarction, which seemed to be caused by spontaneous dissection of the left circumflex coronary artery, and associated papillary muscle disfunction with resultant significant mitral regurgitation. He was treated surgically by coronary artery bypass grafting and mitral valvuloplasty. Ungraftable circumflex was abandoned; instead, pediculated graft of the right gastroepiploic artery was anastomosed to the mid-portion of the right coronary artery, which also had been spontaneously dissected. Mitral regurgitation was treated with Reed's plasty of the posterior commissure and the ring annuloplasty using Carpentier-Edwards Ring. His postoperative course has been smooth and uneventful. According to the English literature of the Western hemisphere, spontaneous coronary arterial dissection usually occurs in relatively young people with a striking predilection for women, and nearly all report deal only with the coronary abnormality. To our knowledge, this is the first report of a surgical case of the spontaneous coronary dissection associated with significant mitral regurgitation.

[Prevention of coronary spasm during and shortly after coronary revascularization in patients with variant angina].

From June 1990 to March 1993, 9 patients undergoing coronary artery bypass grafting (CABG), 4.4% of all CABG cases at our hospital during this period, had significant perioperative coronary spasm. For 4 patients who underwent CABG before May 1992 (Group 1), preventive and suppressive procedures for the coronary spasm were the addition of diltiazem in the cardioplegic solution and the continuous intravenous infusion of nitroglycerin. Perioperative myocardial infarction (PMI) occurred in all 4 patients in Group 1, with the mean peak MB-CPK of 356 +/- 197 IU/l. One patient had delayed sternal closure because of his unstable hemodynamic status. Thereafter, we changed our protocol as follows: 1) Ergometrine loading (intracoronary infusion) test was performed in all candidates for CABG, aiming at finding out patients with a high risk. And for the high-risk patients, in addition to the measures done in Group 1, 2) intraaortic balloon pumping was performed through the perioperative period, and 3) a pig-tail catheter was dwelled in the Valsalva sinus, through which bolus doses of isosorbide dinitrate were injected frequently in this period. 4) Additionally nifedipine was periodically administered through the nasogastric tube. With these intensive preventive/suppressive measures, the perioperative spasm in 5 patients (Group 2) with variant angina were successfully managed, with no resultant PMI nor operative death (The occurrence of PMI was significantly less frequent in Group 2 than in Group 1, with the p value < 0.05). For patients with variant angina undergoing CABG, combined intensive preventive/suppressive measures for perioperative coronary spasm as listed above proved effective.

[A case report of aortic valve replacement after 16 years from repair of ruptured sinus Valsalva aneurysm].

A case of aortic valve replacement after 16 years from the repair of ruptured sinus valsalva aneurysm (RSVA) was reported. The patient has undergone direct closure of RSVA with VSD type I at 34 years old. At the operation, no attempt was made as to aortic valve regurgitation because of small regurgitation, Selloers 1 on aortography. At 50 years old, he developed dyspnea on exertion, to-and-fro murmur due to aortic valve regurgitation, Selloers 3. Aortic valve replacement, we confirmed the completely closure of right coronary sinus valsalva, and histopathologically observed the degenerative change of only right coronary cusp.

[Surgical treatment of ischemic mitral regurgitation].

Mitral regurgitation secondary to ischemic heart disease carries a significant mortality even after emergent open heart surgery. We report on 16 patients with mitral regurgitation associated with ischemic heart disease. The pathological findings were chorda elongation and papillary muscle dysfunction (PMD group) in 12 patients, and papillary muscle rupture (PR group in 4 patients. Preoperative characteristics were severe left ventricular failure in PMD group and high incidence of renal failure, in associated with high age in PR group. In PMD group, mitral plasty was performed in 10 patients out of 12 patients. In PR group all patients underwent mitral valve replacement. Thirteen patients underwent mitral surgery combined with coronary artery bypass grafting. No operative death was seen, one hospital death and 3 late deaths occurred. Three late deaths were seen in PMD group due to sudden death in 2 cases and arrhythmia in 1 case. One hospital death in PR group was due to multi-organ failure. We suggested incorporating these therapeutic concepts may introduce satisfactory results in surgical treatment for ischemic mitral regurgitation.

[Pulmonary hypertension in aortic valve disease].

144 surgical patients with isolated aortic valve disease were reviewed to determine the incidence and the mechanism of pulmonary hypertension (PH). The hemodynamic data revealed PH (pulmonary artery systolic pressure of 40 mmHg or more) in 18 patients (12.6%). There was a positive correlation between left ventricular end-diastolic pressure (LVEDP) and pulmonary artery pressure. After operation the pulmonary artery pressure normalized in all cases but 2 patients in early post-operative period. In patients with aortic stenosis with PH, LVEDP/LVEDVI ratio was higher than that of patients without PH, which showed left ventricular diastolic dysfunction. In patients with aortic regurgitation with PH, the cause of elevated LVEDP was not only severe long-standing regurgitation with ventricular dysfunction, but also the imbalancement of compensation by dilated ventricle with preserved left ventricular function.