Repair of tetralogy of Fallot (TOF) has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS) and free pulmonary regurgitation (PR) at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT) reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR) increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF.
Dilatation ; Heart Failure ; Hemodynamics ; Humans ; Hypertrophy ; Mechanics ; Pulmonary Valve ; Pulmonary Valve Insufficiency ; Pulmonary Valve Stenosis ; Tetralogy of Fallot

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

A valvular perforation is a well-known, and common, complication of infective endocarditis that may adversely affect the clinical outcome. However, a 'windsock' deformity of the mitral valve, as a delayed presentation of infective endocarditis, affecting the mitral valve alone, is very rare. A 42-year-old man, who underwent a mitral valvuloplasty and annuloplasty six years previously, suddenly developed pulmonary edema. He had also had a previous history of infective endocarditis, dating back three years. A transthoracic echocardiogram revealed a 'windsock' deformity of the anterior mitral leaflet (AML), resulting in an acute severe mitral regurgitation. During the operation, the AML was found to have been damaged by the previous endocarditis, resulting in an aneurysmal change of the central scallop, and a rupture of the roof. A mitral valve replacement was successfully performed, and the patient recovered uneventfully. Here, we report a rare case of a 'windsock' deformity of the mitral valve, with two perforations as a delayed complication of a healed infective endocarditis.
Adult ; Aneurysm ; Congenital Abnormalities* ; Endocarditis* ; Humans ; Mitral Valve ; Mitral Valve Insufficiency* ; Pectinidae ; Pulmonary Edema ; Rupture

Severe primary mitral regurgitation (MR) is a progressive condition which engenders significant mortality and morbidity if left untreated. The optimal timing of surgery in patients with MR of degenerative origin continues to be debated, especially for those who are asymptomatic. Apart from symptoms, current authoritative guidelines recommend intervention when there is incipient left ventricular dysfunction, pulmonary hypertension or new onset atrial fibrillation. This review focuses on the asymptomatic subject with severe MR, and examines contemporary clinical decision-making and management strategies, including the 2012 European guidelines on valvular heart disease. We discuss the rationale for risk stratifying the asymptomatic individual, and highlight current and novel diagnostic tools that may have a useful role, with an emphasis on echocardiographic imaging.
Atrial Fibrillation ; Heart Valve Diseases ; Humans ; Hypertension, Pulmonary ; Mitral Valve Insufficiency ; Ventricular Dysfunction, Left

A 38-year-old female patient with a history of tetralogy of Fallot repair at 10 years of age underwent pulmonary valve replacement with a mechanical prosthesis, tricuspid annuloplasty, and right ventricular outflow tract cryoablation due to pulmonary regurgitation, tricuspid regurgitation, and multiple premature ventricular contractions with sustained ventricular tachycardia. After surgery, she had an uneventful postoperative course with arrhythmia monitoring. She was discharged without incident, and a follow-up Holter examination showed a decrease in the number of ventricular ectopic beats from 702 to 41.
Adult ; Arrhythmias, Cardiac ; Cryosurgery* ; Female ; Follow-Up Studies ; Humans ; Prostheses and Implants ; Pulmonary Valve Insufficiency ; Pulmonary Valve* ; Tachycardia, Ventricular ; Tetralogy of Fallot* ; Tricuspid Valve Insufficiency ; Ventricular Premature Complexes

The pressure gradient across the mitral valve and atrioventricular pressure half time were measured by Doppler echocardiography during cardiac catheterization in 15 patients with mitral stenosis. Among these 15 patients with mitral stenosis, 6 patients were combined with aortic insufficiency and 4 patients with mitral insufficiency. Mitral valve area(MVAe) was measured by Doppler echocardiographic pressure half time and mitral valve area(MVAc) was measured by cardiac catheterization data(modified Gorlin's formula). Mean diastolic pressure gradient(MDPG) and peak diastolic pressure gradient(MDPG) measured by Doppler echocaridgraphy were compared with pulmonary wedge pressure. Obtained results were as follows; 1) Thewe was significant correlation between MVAe and MVAc regardless of mitral regurgitation or aortic regurgitation(r=0.09). 2) There was significant correlation between mean diastolic pressure geadient(MDPG) and mean pulmonary wedge pressure(r=0.69). But MDPG were lower than mean pulmonary wedge pressure in patients with mitral regurgitation or aortic regurgitation, which may be due to left ventricular volume overload. 3) The correction coefficiency between peak diastolic pressure gradient(PDPG) measured by Doppler echocardiography and mean pulmonary wedge pressure(r=0.59) was slightly lower than the correlation coefficiency between mean diastolic pressure gradient(MDPG) and mean pulmonary wedge pressure(r=0.69).
Aortic Valve Insufficiency ; Blood Pressure* ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography* ; Echocardiography, Doppler ; Humans ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Mitral Valve* ; Pulmonary Wedge Pressure

BACKGROUND: Ross procedure is the pulmonary valve autograft in the aortic valve disease, and its use trends to increase after introduced by Ross in 1967, firstly. The most important point is that it is a permanent valve replacement. It is to be ideal method to the young patient because the graft is a viable tissue to be able to grow, and hemodynamically, most similar to the normal aortic valve, and doesn't need to do anticoagulation therapy due to not having the thromboembolism, but not popular because it has a lot of technical problem and doesn't have the long-term follow-up METHODS: The patients were 8 admitted between October 1997 and October 1998, the age from 15 to 39 ; 6 males and 2 females. The causes of disease were 4 patients of rheumatic disease, 1 of a infective endocarditis with the aortic annular abscess,1 of recurred severe aortic insufficiency 2 years after replacement. Two patients used the homograft and 6 patients switched a diseased aortic valve with the pulmonary autograft. RESULTS: There were no death and the preoperative dyspnea nearly disappeared (NYHA FC III-IV -> I-II). The diastolic diameter of left ventricle decreased significantly when we compared to the previous echocardiography 1 month after the operation, and we observed the mild aortic valve insufficiency in 3 patients, severe in 4, mild pulmonary valve insufficiency in 4, severe in 1, and mild pulmonary valve stenosis in 4. CONCLUSION: The operative death rate of Ross procedure in the aortic valve disease was not higher than the artificial valve replacement. Therefore, if we find the appropriate indication of operation, we can expect better results and think that we should have the long-term follow-up furthermore.
Allografts ; Aortic Valve ; Aortic Valve Insufficiency ; Autografts* ; Dyspnea ; Echocardiography ; Endocarditis ; Female ; Follow-Up Studies ; Heart Ventricles ; Humans ; Male ; Mortality ; Pulmonary Valve ; Pulmonary Valve Insufficiency ; Pulmonary Valve Stenosis ; Rheumatic Diseases ; Thromboembolism ; Transplantation, Autologous ; Transplants

Isolated tricuspid regurgitation (TR) is rare. Generally, TR is caused by pulmonary hypertension secondary to mitral or aortic valve disease, commonly referred to as "functional" regurgitation. The causes of isolated TR in adults include trauma, endocarditis, carcinoid heart disease, and congenital malformation of the tricuspid valve apparatus. In addition, isolated TR should be distinguished from Ebstein anomaly. In the present case, the patient had no definite causes of TR, and neither mitral nor aortic valve disease. The tricuspid valve of this patient showed no abnormalities other than a severely dilated tricuspid annulus. Isolated TR caused by annular dilatation was diagnosed and then ring annuloplasty was perfomed. The subsequent clinical course was satisfactory.
Adult ; Aortic Valve ; Carcinoid Heart Disease ; Dilatation* ; Ebstein Anomaly ; Endocarditis ; Humans ; Hypertension, Pulmonary ; Tricuspid Valve ; Tricuspid Valve Insufficiency*

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.

PURPOSE: The purpose of this study is to determine whether the new pulmonary valve reconstruction technique prevents short-term postoperative pulmonary regurgitation and improves early and mid-term clinical outcome. METHODS: We reviewed postoperative echocardiographic variables and chest X-ray films from 31 patients who had undergone valve reconstruction(pulmonary valve reconstruction group:PVR) for the repair of TOF between April 2000 and August 2004. We compared the clinical data of these patients with those from 47 patients who had right ventricular outflow tract reconstruction with a monocusp valve(monocusp ventricular outflow patch group:MVOP) and 22 patients who had a transannular patch repair without a monocusp valve(transannular patch group:TAP). RESULTS: In the PVR group, 25 patients(81 percent) had trivial or mild pulmonary regurgitation in their early post operative echocardiogram. Only 12 patients(26 percent) in the MVOP group had mild pulmonary regurgitation; and no patient in the TAP group had it. Pulmonary valve function was good in 96 percent of the PVR group, 36 percent of the MVOP group, and none in the TAP group in early post-operative echocardiogram. Follow-up echocardiogram(1, 2, 3, 4 years later) of the MVOP and TAP groups showed moderate pulmonary regurgitation and severely decreased valve function in almost all cases. However, in the PVR group 54 percent(16/28), 50 percent(14/28), 37 percent(9/24), and 31 percent(5/16) of the patients had trivial or mild pulmonary regurgitation 1, 2, 3 and 4 years after operation, respectively. The valve function remained good in 80 percent(24/30), 64 percent(18/28), 57 percent(12/21), and 31 percent(5/16) of the patients 1, 2, 3 and 4 years after operation respectively. CONCLUSION: Pulmonary valve reconstruction is effective in reducing pulmonary regurgitation and right ventricular dilatation in the repair of TOF, even though regurgitation increases with time. Further study is needed to determine long-term results.
Dilatation ; Echocardiography ; Follow-Up Studies ; Humans ; Pulmonary Valve Insufficiency ; Pulmonary Valve* ; Tetralogy of Fallot* ; Thorax ; X-Ray Film