We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.
Adult ; Azygos Vein ; Dextrocardia ; Drainage ; Heart Defects, Congenital ; Heart Transplantation* ; Heart Ventricles ; Heart* ; Humans ; Isomerism* ; Pulmonary Valve Stenosis ; Veins ; Vena Cava, Inferior ; Vena Cava, Superior

PURPOSE: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. METHODS: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. RESULTS: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). CONCLUSION: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.
Alagille Syndrome* ; Bilirubin ; Cardiovascular Diseases ; Cholestasis ; Cyanosis ; Embryonic Development ; Female ; Heart Diseases ; Hemodynamics ; Humans ; Jaundice ; Labor Presentation ; Liver ; Liver Transplantation ; Mortality ; Pregnancy ; Prognosis ; Pulmonary Valve Stenosis ; Retrospective Studies

We report a case of the isolation of the Aspergillus versicolor complex, initially misidentified by morphological characteristics as the Scopulariopsis species, from a homograft with a bicuspidalized pulmonary valve. An eighteen-month-old female, who had critical pulmonary stenosis, underwent pulmonary valve replacement. On postoperative day 8, she developed a fever, which did not respond to empiric broad-spectrum antibiotics. While no definitive source was identified, a filamentous fungus was isolated from the thawed homograft tissue culture prior to implantation on the operation day. The colonies were powdery green with white edges on Sabouraud dextrose agar. Microscopic examination showed septate hyphae with branched conidiophores and chains of spiny conidia, which suggested Scopulariopsis species. After direct sequencing of the internal transcribed spacer (ITS) regions, the fungus was identified as the A. versicolor complex. To our knowledge, the isolation of the A. versicolor complex from a homograft valve has not been previously described. This case shows that laboratory staff should be aware that microscopic morphology of the A. versicolor complex can resemble that of a number of other genera, including Scopulariopsis species.
Agar ; Anti-Bacterial Agents ; Aspergillus ; Bicuspid ; European Continental Ancestry Group ; Female ; Fever ; Fungi ; Glucose ; Humans ; Hyphae ; Pulmonary Valve ; Pulmonary Valve Stenosis ; Scopulariopsis ; Spores, Fungal ; Transplantation, Homologous

BACKGROUND: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. MATERIALS AND METHODS: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was 79.2+/-14.8 months. RESULTS: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. CONCLUSION: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.
Aortic Valve ; Body Weight ; Cardiopulmonary Bypass ; Constriction, Pathologic ; Follow-Up Studies ; Freedom ; Heart Septal Defects, Ventricular ; Heart Valves ; Humans ; Pulmonary Atresia ; Reoperation ; Retrospective Studies ; Risk Factors ; Survival Rate ; Transplantation, Homologous ; Transplants ; Truncus Arteriosus

The Ross operation, a procedure of replacement of the diseased aortic valve with an autologous pulmonary valve, has many advantages such as no need for anticoagulation therapy and similar valve function and growth potential as native valves. However secondary aortic disease has emerged as a significant complication and indication for reoperation. We report a 48-year-old woman who had Ross operation in 1997 for a damaged bicuspid aortic valve and severe aortic regurgitation due to subacute bacterial endocarditis complicated by aortic root abscess. In 2009, 12 years later, progressive severe aortic regurgitation with incomplete coaptation and mild dilatation of the aortic root was shown on echocardiography and contrasted CT, while the pulmonary homograft retained normal function. She subsequently underwent aortic valve replacement. Histopathological examination of the explanted neo-aortic valve and neo-arterial wall revealed pannus formation at the nodulus Arantii area of the three valve cusps, ventricularis, and arterialis. The amount of elastic fibres in the neo-aorta media was less than usual for an aorta of this patient's age but was similar to a pulmonary artery. The pathological findings were not different from other studies of specimens removed between 7 to 12 years after Ross operation. However, the pathophysiology and long-term implications of these findings remain debatable. Considering the anatomical and physiological changes induced by the procedure, separate mechanisms for aortic dilatation and regurgitation are worthy of consideration.
Aorta/*pathology ; Aortic Valve/*surgery ; Aortic Valve Insufficiency/*etiology ; Cardiovascular Surgical Procedures/*adverse effects ; Dilatation, Pathologic ; Heart Valve Diseases/*surgery ; Prostheses and Implants ; Pulmonary Valve/*transplantation

BACKGROUND: The function of a bioprosthetic heart valve is determined largely by the material properties of the valve cusps. The uniaxial tensile test has been studied extensively. This type of testing, however, does not replicate the natural biaxial loading condition. The objective of the present study was to investigate the regional variability of the biaxial strain versus pressure relationship based on the types of fixation liquid models. MATERIAL AND METHOD: Porcine aortic valves and pulmonary valves were assigned to three groups: the untreated fresh group, the fixed with glutaraldehyde (GA) group, and the glutaraldehyde with solvent (e.g., ethanol) group. For each group we measured the radial and circumferential stretch characteristics of the valve as a function of pressure change. RESULT: Radial direction elasticity of porcine aortic and pulmonary valves were better than circumferential direction elasticity in fresh, GA fixed, and GA+solvent fixed groups (p=0.00). Radial and circumferential direction elasticity of pulmonary valves were better than aortic valves in GA fixed and GA+solvent fixed groups (p=0.00). Radial and circumferential direction elasticity of aortic valves were decreased after GA and GA+solvent fixation (p=0.00), except for circumferential elasticity of GA+solvent fixed valves (p=0.785). The radial (p=0.137) and circumferential (p=0.785) direction of elasticity of aortic valves were not significantly different between GA fixed and GA+solvent fixed groups. Radial (p=0.910) and circumferential (p=0.718) direction of elasticity of pulmonary valve also showed no significant difference between GA fixed and GA+solvent fixed groups. CONCLUSION: When fixing porcine valves with GA, adding a solvent does not cause a loss of mechanical properties, but, does not improve elasticity either. Radial direction elasticity of porcine aortic and pulmonary valves was better than circumferential direction elasticity.
Aortic Valve ; Elasticity ; Glutaral ; Heart Valves ; Pulmonary Valve ; Sprains and Strains ; Transplantation, Heterologous

BACKGROUND: With the advances of cardiac surgery, the demand for an artificial prosthesis has increased, and this has led to the development and utilization of diverse alternative materials. We conducted this research to improve an artificial prosthesis by examining the changes of the physical qualities, the pressure related tensile strength, the change in elasticity and the thermostability of a xenograft valve (porcine) and pericardium (bovine, porcine) based on the type of fixation liquid we used. MATERIAL AND METHOD: The xenograft valves and pericardium were assigned into three groups: the untreated group, the fixed with glutaraldehyde (GA) group and the glutaraldehyde with GA+solvent such as ethanol etc. group. The surgeons carried out each group's physical activities. Each group's uniaxial tension and elasticity was measured and compared. Thermostability testing was conducted and compared between the bovine and porcine pericardium fixed with GA group and the GA+solvent group. RESULT: On the physical activity test in the surgeon's hand, no significant difference between the groups was sensed on palpation. For suture and tension, the GA+solvent group was slightly firmer than the low GA concentration group. In general, the circumferential uniaxial tension and elasticity of the porcine aortic and pulmonary valves were better in the fixed groups than that in the untreated group. There was no significant difference between the GA and GA+solvent groups (p>0.05). Bovine and porcine pericardium also showed no significant difference between the GA group and the GA+solvent group (p>0.05). When comparing between the groups for each experiment, the elasticity tended to be stronger in most of the higher GA concentration group (porcine pulmonary valve, porcine pericardium). On the thermostability testing of the bovine and porcine pericardium, the GA group and the GA+solvent group both had a sudden shrinking point at 80degrees C that showed no difference (bovine pericardium: p=0.057, porcine pericardium: p=0.227). CONCLUSION: When fixing xenograft prosthetic devices with GA, adding a solvent did not cause a loss in pressure-tension, tension-elasticity and thermostability. In addition, more functional solvents or cleansers should be developed for developing better xenografts.
Bioprosthesis ; Elasticity ; Ethanol ; Glutaral ; Hand ; Motor Activity ; Palpation ; Pericardium ; Prostheses and Implants ; Pulmonary Valve ; Solvents ; Sutures ; Tensile Strength ; Thoracic Surgery ; Transplantation, Heterologous

BACKGROUND: Various experimental trials for the development of bioprosthetic devices are actively underway, secondary to the limited supply of autologous and homograft tissue to treat cardiac diseases. In this study, porcine bioprostheses that were treated with glutaraldehyde (GA), ethanol, or sodium dodecylsulfate (SDS) were examined with light microscopy and transmission electron microscopy for mechanical and physical imperfections before implantation. MATERIAL AND METHOD: 1) Porcine pericardium, aortic valve, and pulmonary valve were examined using light microscopy and JEM-100CX II transmission electron microscopy, then compared with human pericardium and commercially produced heterografts. 2) Sections from six treated groups (GA-Ethanol, Ethanol-GA, SDS only, SDS-GA, Ethanol-SDS-GA and SDS-Ethanol-GA) were observed using the same methods. RESULT: 1) Porcine pericardium was composed of a serosal layer, fibrosa, and epicardial connective tissue. Treatment with GA, ethanol, or SDS had little influence on the collagen skeleton of porcine pericardium, except in the case of SDS pre-treatment. There was no alteration in the collagen skeleton of the porcine pericardium compared to commercially produced heterografts. 2) Porcine aortic valve was composed of lamina fibrosa, lamina spongiosa, and lamina ventricularis. Treatment with GA, ethanol, or SDS had little influence on these three layers and the collagen skeleton of porcine aortic valve, except in the case of SDS pre-treatment. There were no alterations in the three layers or the collagen skeleton of porcine aortic valve compared to commercially produced heterografts. CONCLUSION: There was little physical and mechanical damage incurred in porcine bioprosthesis structures during various glutaraldehyde fixation processes combined with anti-calcification or decellularization treatments. However, SDS treatment preceding GA fixation changed the collagen fibers into a slightly condensed form, which degraded during transmission electron micrograph. The optimal methods and conditions for sodium dodecylsulfate (SDS) treatment need to be modified.
Aortic Valve ; Bioprosthesis ; Collagen ; Connective Tissue ; Electrons ; Ethanol ; Glutaral ; Heart Diseases ; Humans ; Light ; Microscopy ; Microscopy, Electron ; Microscopy, Electron, Transmission ; Pericardium ; Pulmonary Valve ; Skeleton ; Sodium ; Transplantation, Heterologous ; Transplantation, Homologous

BACKGROUNDAortic root replacement with pulmonary autograft (Ross procedure) has the advantages of good haemodynamics and growth potential without the need for anticoagulation. In this study, we reviewed our experience of the Ross procedure for patients with aortic valve disease.

METHODSFrom October 1994 to January 2005, 42 Ross procedures were performed in our centre. There were 30 males and 12 females. The mean age was 28 +/- 15 years (range, 5-56 years). Congenital heart disease (CHD) with aortic valve stenosis (AS) and/or aortic valve insufficiency (AI) in 40 cases including one associated with ventricular septal defect (VSD), degenerated aortic valve disease with AS in 1 and subacutive bacterial endocarditis (SBE) with AI in 1 were studied. The diagnosis was made by ultracardiography (UCG) in all patients. The mean aortic valve annulus diameter (AVD) was (2.45 +/- 0.31) cm and pulmonary valve annulus diameter (MPVD) was (2.34 +/- 0.21) cm. All patients had normal pulmonary valves. The New York Heart Association (NYHA) function class was II in 36 cases and III in 6 cases. The operation was performed under moderate hypothermic cardiopulmonary bypass (CPB) with aortic root replacement using pulmonary autograft and pulmonary valve replacement with a homograft.

RESULTSThere was no early hospital mortality. Postoperative UCG showed normal aortic valve function in all our patients. The mean gradient across the aortic valve was (6.11 +/- 0.12) mmHg. The left ventricular diastole diameter (LVDD) decreased significantly from (62 +/- 5) mm to (56 +/- 3) mm (P < 0.001). The mean postoperative left ventricular ejective fraction (LVEF) was 0.49 +/- 0.23. All patients were in NYHA class I-II. Follow-up was completed in 38 cases for a mean period of 3.2 years (range 1-10 years). All survivors were in NYHA class I with normal neo-aortic and pulmonary valve function. One patient died after secondary operation due to homograft fungal endocarditis 1 year after the Ross procedure. The cause of death was uncontrolled bleeding. Another patient suffered from cardiogenic shock and was on extracorporeal membrane oxygenation (ECMO) for 10 days postoperatively. This patient was subsequently self-discharged from hospital due to financial issues and he was excluded from follow-up.

CONCLUSIONThe Ross procedure is an excellent technique to treat aortic valve disease. Our data show that it can be performed safely with good early and mid-term clinical outcomes.

Adolescent ; Adult ; Aortic Valve Insufficiency ; surgery ; Aortic Valve Stenosis ; surgery ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Valve ; transplantation ; Transplantation, Autologous

BACKGROUND: B-type natriuretic peptide (BNP) is a cardiac hormone that is primarily synthesized by the ventricular cardiac myocytes. Increased plasma BNP levels have been observed in patients suffering with congestive heart failure, ventricular hypertrophy and myocaridits and also during heart transplantation rejection. We investigated the serum BNP level as a predictive marker for rejection after heart transplantation. MATERIAL AND METHOD: To test the usefulness of measuring the BNP level in cardiac transplant patients, consecutive blood samplings for BNP, right ventricular endomyocardial biopsies, hemodynamic measurements and transthoracic echocardiogram were all done in 10 such patients between January 2004 and August 2005 at the Department of Thoracic and Cardiovascular Surgery in Asan Medical Center. Two groups were identified with using the median value: the low BNP group (n=28, BNP: < or =290 pg/mL) and the high BNP group (n=29, BNP: >290 pg/mL). We retrospectively analyzed rejection, the ejection fraction, tricuspid regurgitation, left ventricular hypertrophy, the pulmonary capillary wedge pressure and the right atrial pressure between the 2 groups. RESULT: There were no differences in age, gender, rejection, the ejection fraction, tricuspid regurgitation, left ventricular hypertrophy and the right atrial pressure between the 2 groups (p>0.05). However, a higher pulmonary capillary wedge pressure and a higher mean pulmonary atrial pressure were observed in the high BNP group (p<0.05). Further, BNP has linear correlation with the pulmonary capillary wedge pressure (r=0.590, p<0.001). Using the cut-off value of 620 pg/mL, the BNP predicted a high PCWP (>12 mmHg) with a sensitivity of 83.3% and a specificity of 91.1% (AUC: 0.900+/-0.045, p<0.001). CONCLUSION: The BNP level after heart transplantation does not show any significant correlation with rejection, yet it might be a predictive marker of ventricular diastolic dysfunction.
Atrial Pressure ; Biopsy ; Chungcheongnam-do ; Heart Failure ; Heart Transplantation* ; Heart* ; Hemodynamics ; Humans ; Hypertrophy ; Hypertrophy, Left Ventricular ; Myocytes, Cardiac ; Natriuretic Peptide, Brain* ; Plasma ; Pulmonary Wedge Pressure ; Retrospective Studies ; Sensitivity and Specificity ; Tricuspid Valve Insufficiency