Masatsugu Terada

Modified Fontan procedure in ninety-nine cases of atrioventricular valve regurgitation.

Between January 1985 and August 1995, among 242 patients who underwent a modified Fontan procedure, 99 had atrioventricular valve regurgitation ranging in degree from 1 to 4, for which concomitant repair of the atrioventricular valve regurgitation was done in the majority of cases. In all but 4 cases the atrioventricular valve was repaired mainly by circular annuloplasty and valve replacement was not done in any case. Although the hospital mortality rate was significantly higher in cases with atrioventricular valve regurgitation (12/99, 12%) than in cases without (4/143, 3%; p < 0.0037, chi 2 test), actuarial survival in atrioventricular valve regurgitation was 84% for years 5 through 10. The degree of atrioventricular valve regurgitation before operation was 1.6 +/- 0.7 on average: in 49 cases with higher than grade 2 regurgitation before operation there was a significant decrease to 0.4 +/- 0.49 (p < 0.0001) after operation in short-term survivors. Patients with atrioventricular valve regurgitation can be treated with reasonable risk, provided proper repair of the valve is done. Circular annuloplasty is a simple and uniformly effective method to control regurgitation even in cases of common atrioventricular valve.

Hemodynamic Effects of Human Atrial Natriuretic Peptide After Modified Fontan Procedure

BACKGROUND Reduction of pulmonary vascular resistance and maintenance of urine output are important after the modified Fontan procedure. Atrial natriuretic peptide (ANP) has the effects of a vasodilator (including the pulmonary arteries) and a physiologic diuretic, and newly synthesized human ANP is available. We measured plasma ANP levels before and after the Fontan procedure and examined the effects of human ANP on hemodynamic parameters after the Fontan procedure. METHODS Eight patients, aged 2 to 15 years, underwent the Fontan procedure (atriopulmonary connection). Blood samples were taken before and 3 hours after operation, and plasma ANP levels were measured by radioimmunoassay. The correlation between central venous pressure and ANP was examined. Human ANP was infused intravenously at a dosage of 0.1 microg x kg(-1) x min(-1) for 1 hour after the Fontan procedure under controlled ventilation and another blood sample was obtained. Urine volume and central venous pressure were measured, and pulmonary vascular resistance and the cardiac index were calculated by the thermodilution catheter method before and after human ANP infusion. One hour after human ANP infusion was discontinued, the evaluation was repeated. No other diuretics were given and the infusion rates of catecholamine were kept constant during these measurements. RESULTS Plasma ANP levels before and after the Fontan procedure were 29.1 and 54.9 pg/mL, respectively, and a positive correlation was obtained between central venous pressure and plasma ANP levels (r = 0.661, p < 0.05). Human ANP infusion significantly decreased central venous pressure and pulmonary vascular resistance, and increased urine volume and the cardiac index, whereas the plasma ANP level was elevated to 617.5 pg/mL. Systemic blood pressure did not change significantly. CONCLUSIONS Atrial natriuretic peptide is secreted in response to elevated central venous pressure after the Fontan procedure, but its concentration might not be sufficient. Human ANP can be a therapeutic choice after the Fontan procedure as a physiologic diuretic and a pulmonary vasodilator.

Time course of endothelin-1 and adrenomedullin after the Fontan procedure.

BACKGROUND The endothelium-derived vasoconstrictor endothelin (ET)-1 might contribute to the physiology of blood flow regulation and play a role in cardiovascular disease. Adrenomedullin (AM) is a potent vasodilator peptide that has major effects on cardiovascular function and has multiple biologic effects involved in cardiovascular homeostasis. Although pulmonary vascular resistance is known to be one of the most important factors to determine the indications for a Fontan procedure, the time course of the plasma cytokine before and after the Fontan procedure is not known. METHODS Sixteen patients were divided into two groups, 8 patients (1 to 14 years) who had the Fontan procedure (atriopulmonary connection) and 8 age-matched controls who had biventricular repair with normal central venous pressure. Plasma ET-1 and adrenomedullin levels were measured in both groups immediately before cardiopulmonary bypass, immediately after cardiopulmonary bypass, and 6 and 24 hours after cardiopulmonary bypass. A thermodilution catheter was inserted during the operation, and mean pulmonary arterial pressure, pulmonary wedge pressure, and cardiac output were measured, and pulmonary vascular resistance was calculated at the same time points. Correlation between the plasma ET-1 levels and pulmonary vascular resistance data were obtained in the Fontan group. RESULTS Plasma ET-1 levels in the Fontan group were elevated after operation and were higher than the control group at 6 and 24 hours after cardiopulmonary bypass. Plasma adrenomedullin in the Fontan group was lower than in the control group at 6 and 24 hours after cardiopulmonary bypass. A significant positive correlation was obtained between the plasma ET-1 and pulmonary vascular resistance data (r = 0.475). CONCLUSIONS Imbalance between increased ET-1 and relatively decreased adrenomedullin after cardiopulmonary bypass in the Fontan procedure could contribute to dominant effects of ET-1, which might induce vasoconstriction after the Fontan procedure. ET-1 might play an important role in maintaining vasoconstriction after the Fontan procedure.

The long-term results of commissure plication annuloplasty for congenital mitral insufficiency

BACKGROUND Mitral valve repair in the pediatric population remains demanding because of a diversity of apparatus anomalies and the young age of the patients. METHODS We reviewed our clinical results for mitral valve repairs for congenital mitral insufficiency. Forty-nine consecutive patients aged 2 months to 34 years (mean, 4.4 years) had mitral valve repair between June 1984 and December 1996. Forty-one patients (83.7%) had associated cardiac anomalies. The predominant pathologies for the regurgitations were chordal anomalies in 34 patients (69%), annular dilatation in 8 (16%), and leaflet anomalies in 7 (14%). Mitral valve repair included commissure plication annuloplasty in 43 patients (88%), modified DeVega in 11, cleft closure in 5, plication of the anterior leaflet in 3, triangular resection of the anterior leaflet in 2, chordal shortening in 1, and placement of artificial chordae in 1. Several combined techniques were required in 19 patients. RESULTS There were no early or late deaths. The follow-up period was from 6 to 166 months (mean, 88.4 months). Forty-seven patients (95.9%) were in New York Heart Association class I. The long-term echocardiographic studies showed that 2 of 30 patients without reoperation had moderate regurgitation. The actuarial freedom from reoperation was 85.6% (95% confidence limits, 72.8%, 98.4%) at 13 years. Five patients (10.2%) required valve replacement from 13 days to 75 months after the valve repair. Two patients had cerebral ischemic events as a result of cardiomegaly and atrial fibrillation. CONCLUSIONS Valve repair for congenital mitral insufficiency gave adequate results in combination with commissure plication annuloplasty and other techniques with excellent long-term functional status.

Valvular repair for atrioventricular regurgitation in complex anomalies in modified Fontan procedure with reference to a single ventricle associated with a common atrioventricular valve.

Between January 1985 and October 1998, 169 of 372 patients who underwent a modified Fontan procedure had atrioventricular valve regurgitation (AVVR) that ranged in degrees from 1 to 4. Concomitant repair for AVVR was performed in the majority of cases. All but 12 patients had the AV valve repaired, mainly by circular annuloplasty; none had valve replacement. Although mortality was significantly higher in the AVVR cases (21 of 169 [12%]) than in the cases without AVVR (eight of 203 [3.9%]; P <.007, chi-square), actuarial survival in the AVVR cases was 83% at 5 years, 81% at 10 years, and 73% at 12 years. The degree of AVVR before surgery was 1.62 +/- 0.73 on average; 82 cases had more than grade 2 regurgitation. There was a significant decrease to 0.54 +/- 0.61 (P <.0001) after surgery in long-term survivors. Cases with AVVR can be treated with reasonable risk provided proper repair of the valve is performed. Circular annuloplasty is a simple and uniformly effective method with which to control regurgitation, even for the common AV valve. Copyright 1999 by W.B. Saunders Company

Atrial Septal Displacement for Repair of Anomalous Pulmonary Venous Return Into the Right Atrium

BACKGROUND In the repair of anomalous connection of the pulmonary veins to the right atrium, the use of a baffle of pericardium to divert the pulmonary venous blood into the left atrium could cause pulmonary venous obstruction as a result of thickening of the pericardial patch. Anomalous pulmonary venous drainage to the right atrium caused by malposition of the atrial septum primum can be repaired by displacing the shifted septum primum to the normal position. METHODS In 5 patients with total (n=2) or partial (n=3) anomalous pulmonary venous drainage into the right atrium, the septum primum was shifted toward the left atrium and the pulmonary veins drained into the anatomic right atrium despite their normal connection with the posterior wall of the left atrium. This method consisted of incision of the posterior edge of the atrial septum primum and displacement of the incised atrial septum between the anomalous pulmonary veins and both venae cavae. No patch was used. RESULTS Postoperative echocardiography showed a wide pathway from the pulmonary veins to the left atrium with no stenotic portions. No atrial arrhythmias occurred after the operation. CONCLUSIONS This technique may be advantageous because it allows for future growth of the route of the pulmonary venous pathway and avoids postoperative supraventricular arrhythmias.

Fate of Trileaflet Equine Pericardial Extracardiac Conduit Used for the Correction of Anomalies Having Pulmonic Ventricle–Pulmonary Arterial Discontinuity

BACKGROUND External conduits used for the repair of congenital heart diseases having discontinuity between the pulmonic ventricle and the pulmonary artery still carries a high risk of reoperation. Between June 1983 and June 1992, handmade equine pericardial conduit with fabricated trileaflet valve had been the conduit of choice in our institute. The aim of this study is to clarify the temporal sequence of conduit obstruction in this material and to formulate the optimal surgical strategies for this disease entity. METHODS One hundred forty-three patients have undergone extracardiac conduit repair using this conduit. Postoperative catheterization performed within 2 months showed pulmonary to systemic ventricular systolic pressure ratio of 0.57 +/- 0.17 with the pressure gradient between pulmonic ventricle and pulmonary artery of 21.1 +/- 17.2 mm Hg. In 63 patients among the survivors, a series of Doppler two-dimensional echocardiographic images could be clearly obtained. RESULTS Moderate-to-severe degree of pulmonary insufficiency represented only 3.2% of all cases within 3 months, which rapidly increased to 14.3% at 1 to 3 years and 32.8% at 3 to 5 years. However, the rate of increase of pulmonary insufficiency diminished beyond 5 years with 34.9% at 5 to 7 years and 40.0% at 7 to 9 years. Estimated pressure gradient calculated by Bernoullis equation applied in the same patient subset was 4.1 +/- 7.9 mm Hg within 3 months, which progressively increased to 7.1 +/- 11.8 mm Hg at 1 to 3 years, 21.0 +/- 24.0 mm Hg at 3 to 5 years, 40.2 +/- 25.9 mm Hg at 5 to 7 years, and 71.3 +/- 34.0 mm Hg at 7 to 9 years. Among patients with a pressure gradient across the conduit of more than 40 mm Hg at follow-up catheterization, the primary cause of the obstruction was attributed to degeneration of the valve in 7 patients, whereas sternal compression was strongly suspected as the primary cause in the other 8 patients. Intimal peel was not obvious in the excised specimens. CONCLUSIONS Degeneration of the valve in the equine pericardial conduit became prominent at 3 to 5 years after the operation, whereas the pressure gradient across the conduit continued to progress thereafter. A thick and hardened valve from degeneration and varying degrees of external compression by the sternum were delineated at the site of stenosis.

Autologous tissue-fragmented extracardiac conduit with rapid, stable endothelialization due to angiogenesis

OBJECTIVES Autologous angiogenic cytokines are known to activated by mincing stimulation, and well regulated in vivo. We applied this tissue fragmentation technique to a low-pressure pulmonary extracardiac conduit to obtain rapid endothelialization and stable neointima formation due to angiogenesis. METHODS Subcutaneous adipose tissue was obtained, minced, suspended, and sieved through highly porous fabric vascular prosthesis by pressurized injection. The adipose tissue fragmented graft with an autologous fresh pericardial monocusp valve was implanted between the right ventricle and the pulmonary artery in 13 dogs. The same grafts without fragments were implanted in 8 dogs as controls. No anticoagulation therapy was given. RESULTS Grafts were removed 6 to 1,128 days after implantation. In the developed grafts, angiogenesis occurred throughout the interstices of the graft wall from the adventitial side, and host cells proliferated and migrated. Endothelialization was completed throughout developed grafts at 2 weeks. The intima was still thin up to 1,128 days and free of degenerative changes. In control grafts, however, capillary infiltration was limited to perigraft tissue at 2 weeks and endothelialization was not completed by 3 months. Under the endothelial cell layer, laminal elastic fibers were formed through the developed graft wall by 4 months and still maintained at 1,128 days. CONCLUSIONS The results demonstrated that adipose tissue fragmented extracardiac conduits induce rapid endothelialization and maintained thin intima with laminal elastic fibers. Long-term durability is expected based on results from using this technique in a low-pressure pulmonary system in dogs.

Ventricular Hypertrophy as a Risk Factor in Ventricular Septation for Double-Inlet Left Ventricle

BACKGROUND Ventricular septation is an option for surgical correlation of double-inlet or common-inlet left ventricle. However, the surgical risk factors of ventricular septation remain unknown. METHODS Twenty-three patients with double-inlet or common-inlet left ventricle underwent ventricular septation. Preoperative data were compared between the survivors (n = 18) and the nonsurvivors (n = 5) to assess surgical risk factors. RESULTS There were two early deaths (9.5%) and three late deaths (14.3%). Nonsurvivors of ventricular septation were significantly older at the time of operation (14.0 +/- 6.0 versus 7.0 +/- 5.4 years; p < 0.05) and had greater left ventricular mass (383% +/- 100% versus 206% +/- 57% of normal predicted value; p < 0.005) and greater left ventricular mass to left ventricular end-diastolic volume ratio (1.84% +/- 1.18% versus 0.77% +/- 0.17%/% of normal predicted value; p < 0.005). Univariate logistic regression analysis also revealed age at operation (p < 0.05) and mass/end-diastolic volume ratio (p < 0.05) as significant risk factors for death after operation. Multivariate regression analysis revealed that age at operation positively influenced increased mass/end-diastolic volume ratio (p < 0.001). These findings indicated that ventricular hypertrophy was one of the risk factors for ventricular septation, which had a tendency to progress with age. CONCLUSIONS Early operation before progression of ventricular hypertrophy is recommended in patients with double-inlet or common-inlet left ventricle who have suitable anatomy for the ventricular septation procedure.

Konno procedure for congenital aortic stenosis with a single coronary artery from the left coronary sinus.

A right coronary artery originating from the left coronary sinus and traversing anteriorly is thought to be one of the contraindications for a Konno aortoventriculoplasty in congenital aortic stenosis because this procedure necessitates incision of the right ventricular outflow tract. The case of a 5‐year‐old girl with congenital aortic stenosis associated with a single coronary artery, successfully treated surgically by the Konno procedure and right coronary artery reimplantation, is reported. Preoperatively there was a pressure gradient between the left ventricle and the ascending aorta of 109 mmHg, which disappeared postoperatively. A postoperative angiography showed a patent right coronary artery.