Tetralogy of Fallot with absent pulmonary valve is a very rare form of congenital heart disease with various clinical presentations. We experienced a 25-year-old female of severe pulmonary regurgitation due to absent pulmonary valve who had a history of open heart surgery for tetralogy of Fallot and review the literatures.
Adult ; Female ; Heart Defects, Congenital ; Humans ; Pulmonary Valve Insufficiency* ; Pulmonary Valve* ; Tetralogy of Fallot ; Thoracic Surgery

BACKGROUNDPulmonary regurgitation leads to progressive right ventricular dysfunction, susceptibility to arrhythmias, and sudden cardiac death. Percutaneous valve replacement has been developed in recent years, providing patients with an alternative option. Percutaneous pulmonary valve replacement has been recently introduced into clinical practice. The goal of this study was to evaluate the feasibility of percutaneous valve stent insertion to correct the pulmonary regurgitation in sheep using a cup-shaped valve stent.

METHODSPulmonary regurgitation was created by percutaneous cylindrical stent insertion in native pulmonary annulus of 8 sheep. One month after the initial procedure, the sheep with previous cylindrical stent implanted underwent the same implantation procedure of pulmonary valve stent. The valve stent consisted of a cup-shaped stent and pericardial valves. Hemodynamic assessments of the bioprosthetic pulmonary valve were obtained by echocardiography at immediately post-implant and at 2 months follow up.

RESULTSSuccessful transcatheter cylindrical stent insertion was performed in 7 sheep but failed in 1 sheep because the cylindrical stent was released to right ventricle outflow tract. After one month the 7 sheep with pulmonary regurgitation underwent valve stent implantation successfully. Echocardiography confirmed the stents were in desired position during the follow-up. No evidence of pulmonary valve insufficiency occurred in any animals. Echocardiography showed all heart function markers were normal.

CONCLUSIONSPercutaneous cylindrical stent insertion to induce significant pulmonary regurgitation in sheep was feasible, simple and reproducible. Percutaneous pulmonary valve stent implantation can reduce pulmonary regurgitation in a sheep model. Further development of animal model and clinical trials are warranted.

Animals ; Feasibility Studies ; Female ; Heart Valve Prosthesis ; Heart Valve Prosthesis Implantation ; Male ; Pulmonary Valve Insufficiency ; surgery ; Sheep

OBJECTIVETo summarize the experience on auto-pulmonary transplantation (Ross procedure) treating with congenital aortic disease.

METHODSFrom October 1994 to November 2003, 20 cases of Ross procedure were performed to treat with congenital aortic disease, Male: 15 cases; Female: 5 cases; age: 25 years;

DIAGNOSIScongenital heart disease (CHD), aortic abnormalities: 12 cases; aortic valve prolapse: 5 cases; aortic valve hypogenesis: 3 cases; combined with subacute bacterial endocarditis (SBE): 4 cases, and ventricle septal defect (VSD): 2 cases; UCG showed aortic stenosis(AS) and/or aortic insufficience (AI) (moderate to severe), Left ventricle diastole diameter (LVDD): (60.51 +/- 11.87) mm, the grade pressure across aortic valve: (27.04 +/- 6.80) mmHg, heart function (NYHA): Class II: 13 cases; Class III: 3 cases; all cases were performed under CPB and moderate hypothermia, the operation procedure was following: (1) taking off auto-pulmonary artery valve; (2) removing dysfunctional aortic valve and auto-transplantation of pulmonary valve on aortic root; (3) putting a pulmonary homograft to rebuild right ventricular outflow tract.

RESULTSThe mortality was 0 during stay at hospital, aortic valve function were all normal, LVDD decreased significantly (t = 3.4007, P = 0.0008), the grade pressure across aortic valve was in normal limitation, (6.8 +/- 0.19) mmHg. Follow-up showed heart function was in Class I (NYHA), aortic and pulmonary valve function was very well.

CONCLUSIONRoss procedure is a kind of effective alterative operation for treating with congenital aortic valve disease, with good short and middle term results.

Adolescent ; Adult ; Aortic Valve Insufficiency ; surgery ; Aortic Valve Prolapse ; surgery ; Aortic Valve Stenosis ; surgery ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Male ; Pulmonary Valve ; transplantation ; Transplantation, Autologous

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

Cardiac Catheterization ; Cardiac Surgical Procedures ; Heart Valve Prosthesis ; Heart Valve Prosthesis Implantation ; Heart Ventricles/surgery* ; Humans ; Pulmonary Valve/surgery* ; Pulmonary Valve Insufficiency/surgery* ; Treatment Outcome ; Ventricular Outflow Obstruction/surgery*

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: A number of studies have demonstrated that an accurate measurement of the peak tricuspid regurgitation velocity with contimuous wave Doppler, which may predict the right ventricular systolic pressure using the simplified Bernoulli equation. However, the peak velocities are often of low intensity on the spectral Doppler display if the tricuspid regurgitation(TR) is not severe. The aim of this study was to evaluate the usefulness of measuring the time interval from pulmonary closure to the end of tricuspid regurgitation, in predicting peak tricuspid regurgitant velocity and hence derive peak right ventricular systolic and pulmonary artery pressure(PAP). METHODS: We studied 19 patients with normal PAP(group A), and 7 with pulmonary hypertension(group B), 27 with postop state of the open cardiac surgery(group C), who underwent echocardiographic and Doppler assessment. Pulmonary closure(PC) was taken as the closure artifact on the Doppler recording in the main pulmonary artery. Regurgitant flow was identified and recorded in continuous wave mode from the apex. The time interval from end of the TR to PC(TR end PC) was measured, and corrected by 100/min of heart rate. RESULTS: The time interval of corrected “TR and-PC” was 12.95±48.69 msec in group A, 16.8±43.15 msec in group C, where as 72.43±37.71 msec in group B(p < 0.01, comparing with group A). the correlation between TR end-PC and TR gradient was low in group A and C(r=0.12, 0.41 respectively), but high in group B(r=0.73)(TR gradient=0.55×TR end-PC+29.1). CONCLUSIONS: The time interval of TR end-PC in the patients with pulmonary hypertension was higher than normal subjects. And also, we found a good correlation between TR end-PC and TR gradient in patients with pulmonary hypertension. However, in postop state of the open cardiac surgery, there was no significant correlation. The precise explanations of this are by no means clear, but abnormal function of the right ventricle may affect the dynamics of regurgitant flow.
Artifacts ; Blood Pressure ; Echocardiography ; Heart Rate ; Heart Ventricles ; Humans ; Hypertension, Pulmonary ; Pulmonary Artery ; Thoracic Surgery ; Tricuspid Valve Insufficiency

OBJECTIVES: Due to the lack of large-sized pulmonary valved conduit products in clinical practice, hand-sewn expanded polytetrafluoroethylene (ePTFE) valved conduit has been used for right ventricular outflow tract (RVOT) reconstruction in many heart centers around the world. This study aims to summarize the early results of the ePTFE valved conduit and the sewing technology of the conduit in combination with the latest progress, and to provide a reference for the application of ePTFE valved conduit. METHODS: A total of 21 patients using ePTFE valved conduit for RVOT reconstruction in the Second Xiangya Hospital, Central South University from October 2018 to October 2020 were prospectively enrolled in this study. The age at the implantation of the conduit was 4.3 to 43.8 (median 15.1) years old, with weight of (38.9±4.1) kg. In this cohort, 14 patients underwent re-reconstruction of RVOT, including 12 patients with pulmonary regurgitation at 6.3 to 31.0 (median 13.8) years after tetralogy of Fallot (TOF) repair, and 2 patients with failed bovine jugular vein conduit (BJVC). Seven patients underwent Ross operations. Among them, 3 were for aortic valve stenosis, 2 were for aortic regurgitation, and 2 were for both stenosis and regurgitation. The ePTFE valved conduits were standard hand-sewn during the surgery. The 3 leaflets were equal in size with arc-shaped lower edge of the valve sinus. The free edge of the valve leaflets was straight with the length of about 1 mm longer than the diameter. The height of the valve sinus was 4/5 of the diameter. The junction of the valve leaflet was 3/4 of the height of the sinus. The designed leaflets were then continuous non-penetrating sutured into the inner surface of Gore-Tex vessel to make a valved conduit. Valved conduits with diameter of 18, 20, and 22 mm were used in 2, 9, and 10 cases, respectively. The surgical results, postoperative recovery time, and serious complications were summarized, and the changes of postoperative cardiac function status and hemodynamic status of the conduits were investigated. RESULTS: During the implantation of ePTFE valved conduit for RVOT reconstruction, 2 patients underwent mechanical mitral valve replacement with Ross operation, 2 patients with pulmonary regurgitation with repaired TOF underwent left and right pulmonary artery angioplasty, and 1 patient with failed BJVC underwent tricuspid valvuloplasty. The cardiopulmonary bypassing time for patients underwent re-reconstruction of RVOT was (130.9±16.9) min, with aorta clamping for 1 patient to repair the residual defect of the ventricular septum. The cardiopulmonary bypassing and aorta clamping time for Ross operation were (242.7±20.6) min and (145.6±10.5) min, respectively. The duration of postoperative ventilator assistance, intensive care unit stay, and hospital stay were 3.5 h to 7.7 d (median 17.1 h),11.2 h to 29.5 d (median 1.9 d), and 6.0 to 56.0 (median 13.0) d, respectively. All patients survived after discharge from hospital. The follow-up rate after discharge was 100% with median time at 15.0 (13.0 to 39.0) months. No death happened during the follow-up. One patient underwent stent implantation due to right coronary stenosis 2 months after Ross operation. One patient underwent balloon dilation due to right pulmonary artery ostium stenosis 1 year after re-reconstruction of RVOT. The cardiac function of all patients recovered to NYHA class I 6 months after operation. The peak pressure gradient across the valve measured by transthoracic echocardiography before discharge was (9.4±2.6) mmHg (1 mmHg=0.133 kPa), and (18.3±6.1) mmHg at the last follow-up. There was no significant increase in the gradient during the follow-up (P=0.134). No patient suffered from mild or more pulmonary regurgitation. CONCLUSIONS Hand-sewn ePTFE valved conduit is feasible for RVOT reconstruction. It is a promising material for RVOT reconstruction which can effectively meet clinical need. In our experience, the ePTFE valved conduit is simple to manufacture with satisfactory early outcomes.In the application of ePTFE valved conduit, attention should be paid to implantation indications and postoperative anticoagulation management, especially to the preparation details of the valved conduit, to obtain better function and durability of the conduit after implantation.
Adolescent ; Animals ; Cattle ; Constriction, Pathologic/surgery* ; Heart Valve Prosthesis/adverse effects* ; Heart Valve Prosthesis Implantation/methods* ; Humans ; Infant ; Polytetrafluoroethylene ; Prosthesis Design ; Pulmonary Valve Insufficiency/surgery* ; Retrospective Studies ; Treatment Outcome ; Ventricular Outflow Obstruction/surgery*

Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions. For atrial septal defects, an en face view of the tissue defect allows better decisions on device closure. For tricuspid valve malformations, an en face view provides diagnostic information that is difficult to obtain from routine 2D tomography. In repaired tetralogy of fallot with pulmonary regurgitation, preoperative 3D echocardiography- based right ventricular volume may be used to determine the timing of a pulmonary valve replacement in conjunction with cardiovascular magnetic imaging. For optimal adult CHD care, 3D echocardiography is an important complement to routine 2D echocardiography.
Adult* ; Child ; Complement System Proteins ; Echocardiography ; Echocardiography, Three-Dimensional* ; Follow-Up Studies ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Humans ; Pulmonary Valve ; Pulmonary Valve Insufficiency ; Tetralogy of Fallot ; Thoracic Surgery ; Tricuspid Valve

BACKGROUNDAlthough most patients with tetralogy of Fallot undergo radical repair during infancy and childhood, patients that remain undiagnosed and untreated until adulthood can still be treated. This study aimed to evaluate longterm outcomes of adult patients with tetralogy of Fallot who were treated surgically, and to determine the predictors of postoperative pulmonary regurgitation.

METHODSFifty-six adult patients underwent complete surgical repair. Forty-three patients (76.8%) required a transannular patch. Systolic, diastolic, and mean pressure in the main pulmonary artery were measured after repair.

RESULTSThe early mortality rate was 3.6%. The 16-year survival rate was (84.4 ± 11.5)%. Late echocardiography revealed 41 patients with transannular patch who had pulmonary regurgitation, consisting of mild pulmonary regurgitation in 28 patients, moderate in eight, and severe regurgitation in five patients. In addition, there was right ventricular outflow tract stenosis in nine patients, moderate/severe tricuspid valve regurgitation in seven, and residual ventricular septal defect in five. Logistic regression analysis demonstrated that the mean pulmonary pressure measured just after repair predicted late pulmonary regurgitation.

CONCLUSIONSThe long-term survival of surgically treated adult patients with tetralogy of Fallot is acceptable. The mean pressure >20 mmHg in the main pulmonary artery measured right after surgical repair may be a feasible reference to time the reconstruction of the pulmonary valve.

Adolescent ; Adult ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Postoperative Complications ; etiology ; Pulmonary Artery ; physiopathology ; Pulmonary Valve Insufficiency ; etiology ; Survival Rate ; Tetralogy of Fallot ; mortality ; physiopathology ; surgery ; Treatment Outcome