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PubMed1995

Cyanosis and clubbing in a patient with iatrogenic Lutembacher syndrome.

A patient with rheumatic mitral and aortic stenosis is described in whom balloon dilatation of the mitral valve was complicated by an iatrogenic atrial septal defect with a small left to right shunt. Over the course of 4 years, the patient became progressively cyanosed and clubbed and was found to have reversed the shunt across the atrial septal defect due to the occurrence of severe tricuspid stenosis. The tricuspid valve was successfully dilated with re-establishment of a net left to right shunt. This syndrome, the first of its kind, should be appropriately termed the reversed Lutembacher syndrome.

Adolescent↗
PubMedDate not supplied

Case report: trivalvular rheumatic stenosis: documentation of disease progression by serial cardiac catheterization.

A 56-year-old female with known rheumatic tricuspid stenosis was admitted for treatment of palpitations, abdominal discomfort and supraventricular tachyarrhythmias. Twenty years after an initial study, cardiac catheterization disclosed additional mitral and aortic stenoses. Trivalve prosthetic replacement was recommended, but cardiac arrest occurred, allowing pathologic confirmation of the hemodynamic findings. Documentation of disease progression in nonoperated multivalvular rheumatic heart disease by cardiac catheterizations over such extended intervals of time has not previously been described. Also, the clinician is warned that historical and physical signs in such cases can underplay the actual severity and extent of disease, and he should not, therefore, be misled by unimpressive findings that the valvular deformity is insignificant.

Aortic Valve Stenosis↗
PubMed1997

Aortic stenosis in hypoplastic right heart syndrome, associated with interstitial deletion of chromosome 2.

This report describes a rare case of moderate to severe valvular aortic stenosis in the setting of severe tricuspid stenosis, pulmonary atresia with intact interventricular septum and right ventricular dependent coronary circulation. We discuss the clinical, hemodynamic, echocardiographic and angiocardiographic findings on this rare case and comment on accompanied malformations, with its possible association observed to the interstitial deletion of band q22 or q24 of the long arm of chromosome 2.

Abnormalities, Multiple↗
PubMed1992

The vanishing tricuspid annuloplasty. A new concept.

A tricuspid De Vega annuloplasty with absorbable 2-0 polydioxanone sutures was performed on 16 sheep supported by cardiopulmonary bypass. The anulus diameter was reduced by 2 to 6 mm. Four sheep died perioperatively. The 12 survivors were electively killed at 2-week intervals during a period of 6 months. Infective endocarditis was not observed. At the time of death no gradient was found on simultaneous pressure measurements of the right atrium and ventricle. Epicardial echocardiography did not reveal tricuspid stenosis or regurgitation. Macroscopically there was no thrombus and the tricuspid leaflets were of a normal appearance. A thin white cord was observed at the anulus in all animals killed after 12 weeks. The annular size remained as surgically induced until the fourth month, but it reverted to its preoperative size after 5 months. Histologically there was granulation tissue initially only around the foreign material, but then progressively it appeared also between the foreign filaments, with the development of multinucleated foreign body giant cells. At approximately 5 months most of the foreign material had been disrupted or absorbed, leaving a tenuous ring of granulation tissue, which allowed expansion of the tricuspid ring to its original circumference. By 6 months most of the foreign material had disappeared and was replaced by loose collagenous tissue. The concept of a "vanishing annuloplasty" is advanced, with particular application in children and in patients with functional tricuspid insufficiency.

Animals↗
PubMed1997

Incidence and evolution of carcinoid syndrome in the heart.

BACKGROUND AND AIMS OF THE STUDY: Carcinoid syndrome originates from carcinoid tumors localized in the gastrointestinal system, pancreas, biliary vessels, bronchi, ovaries and testes. The condition is characterized by flushing, telangiectasias, diarrhea, bronchoconstriction and cardiac involvement, while cardiac lesions may occur in 50% of patients. This study aimed to investigate-non-invasively-the frequency, severity and evolution of carcinoid heart involvement. METHODS AND RESULTS: Among 87 patients with carcinoid syndrome undergoing routine echocardiography, 39 (45%) had cardiac involvement; 36 of these had thickened, shortened, immobile tricuspid leaflets. Tricuspid regurgitation was present in all 36, it was moderate or severe in 22. Pressure half-time rose to 117 +/- 35 ms, implying mild tricuspid stenosis. The pulmonary valve was also thickened, retracted and immobile with stenotic appearance in 19 patients; regurgitation was present in 16, and mild in 11. Nine patients had pericardial effusion. Of 39 patients with cardiac lesions, 18 had at least one follow up study within 16 +/- 7 months; in 15 cases the valve lesions deteriorated. Tricuspid regurgitation became more severe in six and pressure half-time rose from 107 +/- 46 to 132 +/- 50 ms. Pulmonary regurgitation deteriorated in two, while three patients had new pulmonary valve involvement. The peak gradient across the pulmonary valve increased from 18 +/- 7 to 25 +/- 9 mmHg. Twenty-two patients without heart involvement in the first examination had a follow up study (19 +/- 5 months) during which seven developed valvular lesions, with four moderate and three mild tricuspid regurgitation; mild stenosis was present in all cases. Two patients with mild tricuspid regurgitation had mild mixed pulmonary valve involvement, while of those with moderate tricuspid regurgitation, one also had moderate pulmonary stenosis with mild regurgitation. Five patients with both tricuspid and pulmonary valve diseases had interventional treatment: three underwent balloon valvuloplasty on both valves simultaneously due to stenotic lesions but later relapsed; the other two underwent double valve replacement with biological prostheses but in both cases the pulmonary valve developed severe destruction leading to one death. The other four patients survived for 36, 41, 25 and 16 months, respectively. CONCLUSIONS: Right valvular heart disease occurs frequently in patients with carcinoid syndrome. Evolution of lesions is rapid, leading to right heart failure, though death usually occurs from progressive systemic disease and rarely from pulmonary stenosis. Surgery is the most effective treatment and balloon valvulotomy is only palliative. However, therapy of the systemic condition is predominantly the treatment of choice.

Carcinoid Heart Disease↗
PubMed2000

Congenital Heart Surgery Nomenclature and Database Project: Ebstein's anomaly and tricuspid valve disease.

Ebstein's anomaly is a rare congenital heart defect that is characterized by a spectrum of anatomical abnormalities of the tricuspid valve that also involve the right atrium and right ventricle. The extant nomenclature for Ebstein's anomaly and our approach to the description of the severity of Ebstein's anomaly are reviewed with the objective of establishing a unified reporting system. Although there are common features in Ebstein's anomaly, there is a wide spectrum of pathology with an infinite variety of combinations of severity of the involved structures. An effort was made to develop a classification system that would take into consideration the anatomic abnormalities that help direct the surgical management, particularly in regard to tricuspid valve repair or valve replacement. Isolated congenital tricuspid stenosis and regurgitation are also rare, and a simple classification system is presented. Acquired causes of tricuspid regurgitation and stenosis are more common and are included in the classification system. A comprehensive database set for these malformations is presented so that a comprehensive risk stratification analysis can be performed. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and risk factors are presented.

Databases, Factual↗
PubMed1997

Percutaneous double-valve balloon valvotomy for multivalve stenosis: immediate results and intermediate-term follow-up.

Ten patients each with combined mitral and tricuspid stenosis (group 1) and with combined mitral and aortic stenosis (group 2) underwent double-valve balloon valvotomy as a single staged procedure. The aortic valve was dilated by the Mansfield balloon technique, whereas the mitral and tricuspid valves were dilated with either the Mansfield or Inoue balloon. The mitral valve area increased from 0.78 +/- 0.21 cm2 to 2.05 +/- 0.56 cm2 (p < 0.0005) in group 1 and from 0.75 +/- 0.20 cm2 to 2.1 +/- 0.59 cm2 (p < 0.05) in group 2. The tricuspid valve area increased from 1.11 +/- 0.41 cm2 to 2.52 +/- 0.69 cm2 (p < 0.0005). In group 2, the transaortic gradient decreased from 93.56 +/- 17.7 mm Hg to 28.56 +/- 7.8 mm Hg (p < 0.0005) and the valve area increased from 0.37 +/- 0.05 cm2 to 1.03 +/- 0.25 cm2 (p < 0.005). The excellent symptomatic and hemodynamic results were sustained at 30.3 +/- 9.8 months of follow-up in group 1 and at 23.5 +/- 9.1 months in group 2. Double-valve balloon valvotomy is feasible and safe and provides excellent immediate and intermediate-term follow-up results in selected patients with multivalve disease. A longer follow-up in a larger number of cases is needed to define further the role of this therapy.

Adolescent↗
PubMed1983

The tricuspid valve in carcinoid syndrome. An echocardiographic study.

Carcinoid syndrome was diagnosed in a 48-year-old man by urinary 5-hydroxyindoleacetic acid test results and liver biopsy findings. Cardiac catheterization disclosed tricuspid stenosis and mild pulmonic stenosis. The distinctive echocardiographic findings included a tricuspid valve moving in unison with the annulus and thickened immobile tricuspid leaflets. Unlike in rheumatic heart disease, diastolic doming of the tricuspid leaflets was not seen.

Carcinoid Heart Disease↗
PubMed1996

Traumatic tricuspid insufficiency: a case followed for 32 years, with a note on early sources.

A healthy young man suffered nonpenetrating chest trauma in an automobile accident in August 1962, sustaining tricuspid valve disruption and insufficiency, a rare event. Clinical diagnosis was confirmed by cardiac catherization, but valve replacement did not take place for 10 years. Since initial valve replacement he has had two further operations to deal with valve malfunction resulting in recurrent tricuspid stenosis. He had been followed, in various hospitals, for more than 32 years. Traumatic tricuspid insufficiency has become more common with the rise of car accidents and steering wheel trauma. It is characterized by the subtlety of presentation in its mainly young male victims, although it may be suspected from simple bedside examination, and by the frequently long delay between injury and overt clinical problem. The unusual history of the earliest clinical descriptions is reviewed.

Adult↗
PubMed1991

Tricuspid atresia or severe stenosis with partial common atrioventricular canal: anatomic data, clinical profile and surgical considerations.

The anatomic findings in 11 cases of tricuspid atresia and in two cases of severe tricuspid stenosis, both combined with partial common atrioventricular (AV) canal, are presented in detail. Twelve cases were documented by postmortem examination and the diagnosis was confirmed by echocardiography and surgical observation in the one living patient. Clinical data available in nine cases and cardiac catheterization data obtained in eight are included in this report. In three cases (23%)--two with tricuspid atresia and one with extreme tricuspid stenosis--the tricuspid valve and right ventricle exhibited characteristics seen in Ebstein's anomaly. In all 13 cases, the great arteries were normally related. The ventricular septal defect(s) in 10 (83%) of the 12 postmortem cases rapidly became smaller and this resulted in marked diminution of the pulmonary blood flow and severe hypoxia. Only three of the eight patients with available cardiac catheterization and angiocardiographic data showed the scooped-out appearance of the left ventricular septal surface characteristic of AV canal defects. By contrast, two-dimensional echocardiography, available in the three most recent cases, accurately demonstrated all the defects present and represents the diagnostic method of choice. Early surgical intervention to establish a systemic to pulmonary artery anastomosis is essential for survival. More definitive surgical treatment can be achieved later by an atriopulmonary or cavopulmonary anastomosis with or without replacement of the cleft and often regurgitant mitral valve. The one living patient exemplifies this approach. This is the largest series of this unusual type of tricuspid atresia reported to date.

Cardiac Catheterization↗
PubMed1991

Long-term function in University of Cape Town prostheses in the tricuspid position.

A 55-year-old woman undergoing triple-valve replacement was found at operation to have a well preserved and functioning University of Cape Town prosthesis that had been inserted 18 years earlier for tricuspid stenosis. Case reporting, valve reappraisal, and factual updating on another case, the world's first documented tricuspid valve replacement for Ebstein's anomaly, are presented. These two remarkable in vivo successes speak well for this historic collar stud valve.

Female↗