Long-Term Comparison of Three Types of Aortic St. Jude Medical Mechanical Prosthesis in Japanese Patients.

BACKGROUND: The long-term results achieved with aortic St. Jude Medical (SJM) mechanical prostheses in various age groups of Japanese patients have not been previously compared or reported. METHODS AND RESULTS: Since 1981, a total of 240 SJM valves were implanted in 79 patients using the Standard model, in 58 patients with the Hemodynamic Plus model, and in 103 patients with the Regent model for aortic valve replacement (AVR). Follow-up was completed for 2,397 patient-years in 97.5% of the patients, among whom the effect of age was compared, and the subjects were divided into younger (<65 years) and older (≥65 years) groups. Hospital mortality rate was 2.5%. No structural valve deterioration was observed during the follow-up period. In addition, no significant differences were observed in long-term survival between the 3 models. In contrast, significantly better rates of freedom from all-cause death (P<0.0001), valve-related death (P=0.0018) and valve-related morbidity (P=0.0021), including bleeding events (P=0.0007), were observed in the younger group (n=157, 50.6±1.0 years old) than in the older group (n=83, 72.5±0.7 years old). CONCLUSIONS: All types of SJM valve used for single AVR achieved satisfactory early and long-term results in each age group even 25 years after surgery. When selecting this prosthesis for elderly patients, however, relatively worse performance may be expected compared with that observed in younger patients.

More than 20-year experience of Bentall operation with mechanical prostheses for chronic aortic root aneurysm.

OBJECTIVES: Long-term results of Bentall operations with mechanical prostheses were analyzed and evaluated over 30 years. METHODS: Seventy-one patients aged 50.2 ± 1.8 years old underwent an elective Bentall operation from 1975 to 2013 for chronic aortic root aneurysm, while 129 patients aged 51.6 ± 1.3 underwent isolated AVR with a mechanical valve for pure aortic regurgitation. The follow-up was completed for a total of 2,336-patient-years in 99.5% of these patients. RESULTS: No significant differences were observed in the hospital mortality (2.8 and 0.78%), actuarial survival rate (43.3 ± 9.9 and 50.0 ± 9.2%), freedom from valve-related death (84.9 ± 6.8 and 68.1 ± 11%), and freedom from valve-related morbidity (43.7 ± 19 and 40.1 ± 15%) at 30 years between the two groups. No significant differences were observed in the valve-related events; however, a higher incidence of rupture of aortic aneurysm was observed in the Bentall group (P = 0.0005). CONCLUSIONS: Both our short- and long-term results of Bentall operation with mechanical prostheses were satisfactory at 30 years after the surgery and were comparable with those of simple AVR. However, to prevent rupture of the aortic aneurysm, special care should be taken after the primary Bentall operations.

Long-term results of aortic valve replacement with mechanical prosthesis or carpentier-edwards perimount bioprosthesis in Japanese patients according to age.

BACKGROUND: The long-term results of aortic valve replacement (AVR; n=737) with bileaflet mechanical prosthesis (MP) or Carpentier-Edwards Perimount bioprostheses (BP) were evaluated in different age groups. METHODS AND RESULTS: Since 1981, a total of 737 prostheses (424 bileaflet MP vs. 313 BP) were implanted for AVR in 278 patients aged ≥70 years (79 MP vs. 199 BP), in 191 patients aged 60-69 years (128 MP vs. 63 BP) and in 268 patients aged <60 years (217 MP vs. 51 BP). Follow-up was completed for 6,523 patient-years in 98.5% of cases. Among the patients ≥70 years, both the actuarial survival rate (P=0.0434) and freedom from valve-related morbidity (P=0.0205) were better in the BP group than in the MP group without any difference in occurrence of structural valve deterioration in both groups. Among the patients aged 60-69, anticoagulant-related complications occurred less often in the BP group (P=0.0134) without any difference in long-term survival. Among the patients aged <60, long-term survival was significantly better in the MP group, whereas freedom from anticoagulant-related events did not differ. CONCLUSIONS: The use of BP is suitable in patients aged ≥70 years, while the use of bileaflet MP is preferable in patients aged <60 years. Among patients aged 60-69 years, the use of BP is acceptable because of the lower incidence of anticoagulant-related events and the equivalent long-term survival.

Quantitative assessment of right ventricular function and pulmonary regurgitation in surgically repaired tetralogy of Fallot using 256-slice CT: comparison with 3-Tesla MRI.

OBJECTIVES: To compare 256-slice cardiac computed tomography (CCT) with cardiac magnetic resonance (CMR) imaging to assess right ventricular (RV) function and pulmonary regurgitant fraction (PRF) in patients with repaired tetralogy of Fallot (TOF). METHODS: Thirty-three consecutive patients with repaired TOF underwent retrospective ECG-gated CCT and 3-Tesla CMR. RV and left ventricular (LV) end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) were measured using CCT and CMR. PRF-CCT (%) was defined as (RVSV - LVSV)/RVSV. PRF-CMR (%) was measured by the phase-contrast method. Repeated measurements were performed to determine intra- and interobserver variability. RESULTS: CCT measurements, including PRF, correlated highly with the CMR reference (r = 0.71-0.96). CCT overestimated RVEDV (mean difference, 17.1 ± 2.9 ml), RVESV (12.9 ± 2.1 ml) and RVSV (4.2 ± 2.0 ml), and underestimated RVEF (-2.6 ± 1.0%) and PRF (-9.1 ± 2.0%) compared with CMR. The limits of agreement between CCT and CMR were in a good range for all measurements. The variability in CCT measurements was lower than those in CMR. The estimated effective radiation dose was 7.6 ± 2.6 mSv. CONCLUSIONS: 256-slice CCT can assess RV function and PRF with relatively low dose radiation exposure in patients with repaired TOF, but overestimates RV volume and underestimates PRF. KEY POINTS: 256-slice CT assessment of RV function is highly reproducible in repaired TOF. Pulmonary regurgitation can be evaluated by biventricular systolic volume difference. CT overestimates RV volume and underestimates pulmonary regurgitation, compared with MRI.

Staphylococcal endocarditis as the first manifestation of heritable protein S deficiency in childhood.

A 12-year-old Japanese girl developed infective endocarditis and central nervous system disease. The previously healthy girl showed altered consciousness and abnormal behaviors along with the classical signs of septic emboli. Staphylococcus aureus was isolated from peripheral blood, but not, the pleocytotic cerebrospinal fluid. Diagnostic imaging studies revealed a vegetative structure in the morphologically normal heart, and multiple thromboembolisms in the brain and spleen. Low plasma activity of protein S (12%) and thrombophilic family history allowed the genetic study, demonstrating that she carried a heterozygous mutation of PROS1 (exon 13; 1689C > T, p.R474C). Surgical intervention of the thrombotic fibrous organization and subsequent anticoagulant therapy successfully managed the disease. There are no reports of infective endocarditis in childhood occurring as the first presentation of heritable thrombophilia. Protein S deficiency might be a risk factor for the development or exacerbation of infective endocarditis in children having no pre-existing heart disease.

Single-institution, 22-year follow-up of 786 CarboMedics mechanical valves used for both primary surgery and reoperation.

OBJECTIVES: The long-term (>20 years) results for CarboMedics mechanical valves (Sorin Group, Milano, Italy) used for both primary surgery and reoperation have never been reported or compared. METHODS: Since 1990, a total of 787 CarboMedics valves have been implanted in 694 patients for aortic valve replacement, including 19 redo cases in 220 patients; for mitral valve replacement, including 108 redo cases in 381 patents; and for double (aortic and mitral) valve replacement, including 29 redo cases in 93 patients. The follow-up data were complete for 7201 patient-years in 99.3% of the patients. RESULTS: The hospital mortality rate of the aortic, mitral, and double valve replacement groups was 0.9%, 3.7%, and 4.3%, respectively. The corresponding freedom from valve-related morbidity rates in each group were 66.0%, 40.6%, and 48.0% at 20 years (P = .0206). A higher incidence of paravalvular leakage was observed in the mitral and double valve replacement groups than in the aortic valve replacement group (P = .0019). Of the cases of mitral paravalvular leakage after single mitral valve replacement, 97% occurred after redo single mitral valve replacement; 73% of the cases of mitral paravalvular leakage after double valve replacement occurred after redo double valve replacement. CONCLUSIONS: CarboMedics mechanical valves used for both primary surgery and reoperation for aortic, mitral, and double valve replacement can achieve satisfactory early and long-term results, even 20 years after surgery. Care should be taken, however, to prevent paravalvular leakage in the mitral position during reoperation.

The novel EuroSCORE II algorithm predicts the hospital mortality of thoracic aortic surgery in 461 consecutive Japanese patients better than both the original additive and logistic EuroSCORE algorithms.

OBJECTIVES: The European System for Cardiac Operative Risk Evaluation (EuroSCORE) II was developed to improve the overestimation of surgical risk associated with the original (additive and logistic) EuroSCOREs. The purpose of this study was to evaluate the significance of the EuroSCORE II by comparing its performance with that of the original EuroSCOREs in Japanese patients undergoing surgery on the thoracic aorta. METHODS: We have calculated the predicted mortalities according to the additive EuroSCORE, logistic EuroSCORE and EuroSCORE II algorithms in 461 patients who underwent surgery on the thoracic aorta during a period of 20 years (1993-2013). RESULTS: The actual in-hospital mortality rates in the low- (additive EuroSCORE of 3-6), moderate- (7-11) and high-risk (≥11) groups (followed by overall mortality) were 1.3, 6.2 and 14.4% (7.2% overall), respectively. Among the three different risk groups, the expected mortality rates were 5.5 ± 0.6, 9.1 ± 0.7 and 13.5 ± 0.2% (9.5 ± 0.1% overall) by the additive EuroSCORE algorithm, 5.3 ± 0.1, 16 ± 0.4 and 42.4 ± 1.3% (19.9 ± 0.7% overall) by the logistic EuroSCORE algorithm and 1.6 ± 0.1, 5.2 ± 0.2 and 18.5 ± 1.3% (7.4 ± 0.4% overall) by the EuroSCORE II algorithm, indicating poor prediction (P < 0.0001) of the mortality in the high-risk group, especially by the logistic EuroSCORE. The areas under the receiver operating characteristic curves of the additive EuroSCORE, logistic EuroSCORE and EuroSCORE II algorithms were 0.6937, 0.7169 and 0.7697, respectively. Thus, the mortality expected by the EuroSCORE II more closely matched the actual mortality in all three risk groups. In contrast, the mortality expected by the logistic EuroSCORE overestimated the risks in the moderate- (P = 0.0002) and high-risk (P < 0.0001) patient groups. CONCLUSIONS: Although all of the original EuroSCOREs and EuroSCORE II appreciably predicted the surgical mortality for thoracic aortic surgery in Japanese patients, the EuroSCORE II best predicted the mortalities in all risk groups.

Regulation of human autoimmune regulator (AIRE) gene translation by miR-220b.

Although mutations of autoimmune regulator (AIRE) gene are responsible for autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), presenting a wide spectrum of many characteristic and non-characteristic clinical features, some patients lack AIRE gene mutations. Therefore, something other than a mutation, such as dysregulation of AIRE gene, may be a causal factor for APECED or its related diseases. However, regulatory mechanisms for AIRE gene expression and/or translation have still remained elusive. We found that IL-2-stimulated CD4(+) T (IL-2T) cells showed a high expression of AIRE gene, but very low AIRE protein production, while Epstein-Barr virus-transformed B (EBV-B) cells express both AIRE gene and AIRE protein. By using microarray analysis, we could identify miR-220b as a possible regulatory mechanism for AIRE gene translation in IL-2T cells. Here we report that miR-220b significantly reduced the expression of AIRE protein in AIRE gene with 3'UTR region transfected 293T cells, whereas no alteration of AIRE protein production was observed in the open reading frame of AIRE gene alone transfected cells. In addition, anti-miR-220b reversed the inhibitory function of miR-220b for the expression of AIRE protein in AIRE gene with 3'UTR region transfected cells. Moreover, when AIRE gene transfected cells with mutated 3'UTR were transfected with miR-220b, no reduction of AIRE protein production was observed. Taken together, it was concluded that miR-220b inhibited the AIRE gene translation through the 3'UTR region of AIRE gene, indicating that miR-220b could serve as a regulator for human AIRE gene translation.

Mechanical prosthesis is reasonable for mitral valve replacement in patients approximately 65 years of age.

BACKGROUND: The long-term results of mitral valve replacement (MVR; n = 631) with a bileaflet mechanical prosthesis or a Carpentier-Edwards Perimount bioprosthesis were evaluated in Japanese patients of different age groups. METHODS: A total of 507 bileaflet mechanical prostheses and 124 bioprostheses have been implanted since 1982 at our institution. Follow-up was completed for 6,598 patient-years in 98.4% of the cases. RESULTS: Among the patients 70 years of age and older, the rate of freedom from valve-related death and valve-related morbidity at 10 years after surgery were significantly better in the bioprostheses group (93.3% ± 6.4% and 83.7% ± 8.7%, respectively; n = 35) than in the mechanical prostheses group (71.1% ± 8.0% and 60.9% ± 8.9%, respectively; n = 82), and neither structural valve deterioration (SVD) nor resulting re-MVR were observed for bioprostheses. In contrast, among the patients 64 years and younger, no significant differences were observed in long-term survival between the mechanical prostheses group (n = 347) and the bioprostheses group (n = 76), while significantly lower rates of freedom from SVD and re-MVR were observed in the bioprostheses group compared with those obtained in the mechanical prostheses group. As for the controversial intermediate-age group of 65 to 69 years, the general tendencies were similar to those observed in the group 64 years and younger. CONCLUSIONS: Based on our comparative evaluation, bioprostheses should be chosen for MVR in patients 70 years of age and older, whereas mechanical prostheses were better in the patients 64 years of age and younger. The use of bioprostheses in Japanese patients 65 to 69 years of age is not preferable for preventing SVD and subsequent re-MVR.

Left ventricular efficiency after ligation of patent ductus arteriosus for premature infants.

OBJECTIVE: The purpose of this study was to evaluate the hemodynamic changes in left ventricular function before and after patent ductus arteriosus ligation in premature infants with regard to the energetic efficiency of left ventricular pumping. METHODS: Thirty-five premature infants who underwent patent ductus arteriosus ligation were enrolled in this study. Left ventricular efficiency was evaluated at 4 points: within 24 hours before patent ductus arteriosus ligation, within 24 hours after patent ductus arteriosus ligation, between postoperative days 2 and 4, and on postoperative day 7. The indices of contractility (end-systolic elastance) and afterload (effective arterial elastance) were approximated on the basis of the systemic blood pressure and systolic or diastolic left ventricular volume. The ratio of stroke work and pressure-volume area, representing the ventricular efficiency, was estimated using the following theoretic formula: the ratio of stroke work and pressure-volume area = 1/(1 + 0.5 ventriculoarterial coupling). RESULTS: Left ventricular efficiency was transiently deteriorated within 24 hours after patent ductus arteriosus ligation because of the marked increase of the afterload and the slight increase of contraction, and then recovered to preoperation levels by 2 to 4 days after patent ductus arteriosus ligation. CONCLUSIONS: Analysis of indices representing the afterload, contractility, and energetic efficiency of the left ventricle may provide practical information for the management of premature infants during the postoperative period after patent ductus arteriosus ligation.

Left atrioventricular valve regurgitation after correction of atrioventricular septal defects.

PURPOSE: Progressive regurgitation of the left atrioventricular valve (AV) remains a major postoperative problem in the repair of atrioventricular septal defect (AVSD). The aim of this study was to review a case series of AVSD repair and reoperation for significant left AV valve regurgitation from this institution. METHODS: Forty-nine patients underwent initial repair of AVSD between February 1990 and March 2011, and 4 of them underwent reoperation for left AV valve regurgitation. Another 5 patients, who had received initial repair of AVSD before 1990, underwent reoperation of the left AV valve during the same period. This study retrospectively reviewed all cases of AVSD operation, and considered the causes of the left AV valve incompetence, and furthermore addressed how to manage most effectively this problem. RESULTS: There were 4 early deaths (8.6%) and no late deaths after initial repair of AVSD. No death was observed after reoperation of the left AV valve. Six patients underwent re-repair of left AV valve, but three patients needed prosthetic valve replacement. An additional cleft closure and commissuroplasty were performed on the 6 re-repaired cases. No significant AV valve regurgitation was observed among the 6 re-repaired cases. The actuarial survival was 92% at 10 and 15 years after AVSD repair. Freedom from reoperation of the left AV valve was 81% at 10 and 15 years for the patients who survived the initial repair during the study period. Freedom from significant left AV valve regurgitation was 46% at 10 years for all patients who survived the initial repair during the study period. A partial ring annuloplasty using Gore-Tex graft was applied to the last 2 cases, and this employment yielded encouraging results. CONCLUSION: The results were acceptable in terms of the mortality and reoperation free ratio, but the freedom from significant left AV valve regurgitation was disappointing. The outcome of reoperation for significant left AV valve regurgitation was also satisfactory. The application of Gore-Tex graft partial annuloplasty of the left AV valve appears to be a potentially useful and effective treatment modality.

Pulmonary valve replacement long after repair of tetralogy of Fallot.

PURPOSE: Pulmonary valve replacement long after repair of tetralogy of Fallot can improve cardiac function, functional status, and arrhythmia propensity. This has not been reported in Japan. We aim to evaluate the effects of pulmonary valve replacement in repaired tetralogy of Fallot. METHODS: Nineteen patients underwent pulmonary valve replacement after repair of tetralogy of Fallot, excluding Rastelli type operation, between August 1981 and August 2011. The results of the pulmonary valve replacement were assessed by analyzing preoperative and postoperative cardiothoracic ratio, cardiac function, functional status, QRS duration and durability of the prosthetic valves. RESULTS: There were neither operative nor late deaths. The Cardiothoracic ratio significantly improved from 61.0 ± 5.2 % preoperatively to 56.2 ± 4.8 % postoperatively (P < 0.001). The New York Heart association functional class significantly improved from 2.4 ± 0.8 preoperatively to 1.2 ± 0.4 postoperatively as well. Left ventricular ejection fraction showed significant improvement. QRS duration showed significant reduction. The freedom redo pulmonary valve replacement at 20 years was 100 %. CONCLUSION: Pulmonary valve replacement long after repair of previous tetralogy of Fallot had clinical benefits with low mortality. We recommend bioprosthesis for pulmonary valve replacement when adult-sized valve can be accommodated.

Successful surgical treatment for methicillin-resistant Staphylococcus aureus endocarditis on the ventricular rerouting patch after a Rastelli operation.

We report a very rare case of successful surgical treatment for methicillin-resistant Staphylococcus aureus endocarditis on the Dacron patch utilized for ventricular rerouting in a Rastelli operation. Vegetations were found on the right side of the patch associated with a large laceration. The patient underwent removal of the patch followed by reventricular rerouting and replacement of the right ventricle to pulmonary artery conduit. The postoperative course was uneventful. To the best of our knowledge, this is the first reported case of postoperative infective endocarditis on this location.

Advantages of the L-incision approach comprising a combination of left anterior thoracotomy and upper half-median sternotomy for aortic arch aneurysms.

Although surgical outcomes of total arch replacement have improved, the strategy for extended arch aneurysms remains controversial. We have applied the L-incision approach (combination of left anterior thoracotomy and upper half-median sternotomy) for total arch replacement for single-stage repair of extensive arch aneurysms. We retrospectively reviewed the operative outcomes of patients who underwent total arch or extended total arch replacement for degenerative aneurysms from 1999 to 2010. Operations were performed via median sternotomy in 47 patients (M group) and the L-incision approach was used in 38 patients (L group). Through the L-incision approach, we were able to complete distal anastomosis below the pulmonary hilus. The L-incision approach has advantages of reducing selective antegrade cerebral perfusion and lower body circulatory arrest times compared with the M group. Recurrent laryngeal nerve palsy and renal dysfunction were less frequent in the L group than those in the M group. Respiratory dysfunction and wound infection were similar between the groups. Hospital mortalities were 5.3% in the L group and 6.4% in the M group. The L-incision approach has similar or better postoperative outcomes compared with the median sternotomy approach. This approach could be useful for single-stage extended total arch replacement with relatively low risk.

Surgical repair of an idiopathic pulmonary artery aneurysm.

We report a rare case of idiopathic pulmonary artery aneurysm (PAA) in a 56-year-old woman without any causative conditions, such as congenital heart disease, inflammation, pulmonary artery hypertension, or systemic vasculitis. She presented with sudden back pain, and examination revealed the PAA. She electively underwent resection of the aneurysm and graft replacement. Pathology examination revealed cystic medial necrosis, which was considered the underlying pathology of the aneurysm.

Early in vivo evaluation of ventricular assistance with a miniature centrifugal blood pump (TinyPump) in rabbits.

We have developed an ultraminiature centrifugal pump, TinyPump, with a priming volume of 5 ml. The in vivo performance of TinyPump was compared with that of HPM-05 for left ventricular support. Each pump group included seven rabbits weighing 3.4-3.8 kg. One rabbit in the TinyPump group and two rabbits in the HPM-05 group died of unsuccessful cannulation. The remaining rabbits (six in the TinyPump group and five in the HPM-05 group) were instrumented and observed for 240 minutes. The pump flow was maintained at around 200 ml/min. The priming volumes of the entire circuits were 25 and 45 ml for TinyPump and HPM-05, respectively. TinyPump required a higher rotation speed (2214 +/- 47 vs. 1261 +/- 87 rpm, p < 0.05) because of its small priming volume but showed a similar plasma free hemoglobin level to HPM-05. The hematocrit values were kept higher in the TinyPump group during ventricular support (24.3 +/- 1.4% vs. 20.1 +/- 1.4% at 240 minutes, p < 0.05). The mean arterial pressure did not differ between the two groups. The biochemical parameters were also equivalent in the two groups. Overall, TinyPump exhibited a feasible in vivo performance. This ultraminiature device would offer promising outcomes for neonates and infants with intractable heart failure.

Obstructive prosthetic atrioventricular valve thrombosis in a woman with congenitally corrected transposition of the great arteries during estrogen replacement therapy for pituitary dysfunction.

A 27-year-old woman was admitted because of breathlessness, orthopnea, and hemoptysis. The present patient was diagnosed with congenitally corrected transposition of the great arteries (cc-TGA) and underwent systemic atrioventricular valve replacement for severe insufficiency at 23 years of age. She also had been treated with oral conjugated equine estrogen (Premarin) because of congenital pituitary dysfunction. Despite appropriate anticoagulation therapy with warfarin, echocardiography and fluoroscopy showed stuck leaflets of the prosthetic valve due to thrombosis. She underwent emergent surgical valve replacement. This rare association suggests that oral hormone replacement therapy poses a risk of thrombosis especially in patients with cc-TGA after prosthetic valve replacement.

Para-Hisian pacing for a pediatric patient with a congenitally corrected transposition of the great arteries (SLL).

We report a pediatric patient with a congenitally corrected transposition of the great arteries (ccTGA)(SLL) in which permanent para-Hisian pacing (PPHP) could improve dyssynchrony-associated systemic ventricular (SV) dysfunction resulting from permanent morphologic left ventricular pacing for complete atrioventricular block. Since, in patients with ccTGA(SLL), an elongated His-bundle runs medially toward the upper septum to the site of the fibrous continuity between the right-sided mitral valve and pulmonary artery, the His-bundle may easily be captured by a pacing lead, unlike in normal hearts. Thus, PPHP may be an effective therapeutic strategy for the treatment of dyssynchrony-associated SV dysfunction associated with ccTGA (SLL).

A novel SCN5A mutation associated with the linker between III and IV domains of Nav1.5 in a neonate with fatal long QT syndrome.

A male newborn weighing 2334 g was delivered at 37 weeks of gestation by caesarean section because of prenatal ultrasound findings of fetal hydrops with atrioventricualr block, ventriucular tachycardia (VT), and impaired ventricular function. In spite of the intravenous administration of lidocaine, VT continued. He developed poor perfusion and systemic hypotension. After the intravenous administration of amiodarone, VT was terminated. The electrocardiogram revealed an extremely prolonged corrected QT interval (860 ms) with 2:1 atrioventricular block. Unfortunately, he died 18 h after birth in spite of the administration of lidocaine, beta-blocker and magnesium. Mutational analysis identified a novel heterozygous de novo mutation (F1486del) in SCN5A. This mutation is associated with the IFM motif in the linker between III and IV domains of Na(v)1.5, which serves as an inactivation particle binding within the pore of sodium channels. This report demonstrates an interesting relationship between the clinical phenotype and the location of the mutation in long QT syndrome.

The somatic growth of autologous vessels in venous pathway after extracardiac total cavopulmonary connection.

OBJECTIVE: In a total cavopulmonary connection (TCPC) with an extracardiac conduit, the future development of stenosis in the venous pathway and distortion of the pulmonary artery according to the somatic growth of the patients is a major concern for surgeons and pediatricians. METHODS: Thirty patients who underwent extracardiac TCPC (EC-TCPC) between 1990 and 1998 and who had received at least two postoperative angiograms were enrolled in this study. To evaluate the postoperative change in the anastomosis, the cross-sectional area of the venous root at three different points was measured on the first and second angiograms after the EC-TCPC. Further, to evaluate the somatic growth of the autologous tissue, 12 patients who grew more than 10 cm in height after the completion of the EC-TCPC were selected among the 30 patients. We measured the length of three different parts, the diameter of the pulmonary artery, length of the artificial graft, and length between the branching point of the hepatic vein and artificial graft's anastomotic site to the inferior vena cava. RESULTS: The cross-sectional area at each point did not change during the follow-up, and the pressure gradient across the grafts has not been observed. The diameter of the pulmonary artery and length of the inferior vena cava above the hepatic vein insertion grew similarly during the follow-up. CONCLUSIONS: The mid-term clinical results after the completion of the EC-TCPC, including the somatic growth of the venous pathway, were satisfactory.