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PubMed2000

Tricuspid valve repair: a rational alternative.

BACKGROUND AND AIMS OF THE STUDY: The prosthetic ring annuloplasty and incompletely encircling suture techniques are effective methods of tricuspid valve repair when the problem is only annular dilatation, but not when organic tricuspid valve disease is present. A surgical technique of valve repair has been developed that is equally effective in correcting purely functional as well as organic valvular incompetence. METHODS: The Manipal method of repairing the incompetent tricuspid valve consists of three steps: (i) anteroseptal commissurotomy and asymmetric 'U-on-side' suture annuloplasty, to push the plane of coaptation of the anterior and septal leaflets into the right ventricle; (ii) a semicircular De Vega-type of plicating suture through the annulus, starting and ending just cephalad to the posteroseptal commissure and extending anticlockwise to a point just caudal to the meridian, to exclude the posterior leaflet; and (iii) tying the plicating suture after positioning a 3M Starr-Edward valve sizer across the tricuspid valve (in an adult), to ensure that the valve orifice is not excessively narrowed. RESULTS: Between July 1986 and January 1997, the Manipal method was used to repair 52 tricuspid valves, always combined with surgery for the mitral and/or aortic valve. Tricuspid stenosis of varying degree was present in 61% of cases. One of two hospital deaths was related to the repaired valve. Although the proportion of patients followed up fell progressively to 33% at 10 years, none of the patients either seen personally or who had replied to a postal questionnaire (78% of total patients) required reoperation for valve regurgitation or obstruction. No patient had more than mild tricuspid regurgitation clinically, even seven and 10 years after tricuspid valve repair surgery. CONCLUSION: This alternative method of tricuspid valve repair is simple to execute, is equally effective in correcting both pure tricuspid regurgitation and organic tricuspid valve disease, and appears to be extremely stable.

Adolescent↗
PubMed1978

Hemodynamic studies after De Vega's tricuspid annuloplasty.

Postoperative hemodynamic studies were conducted at rest and during exercise in 24 patients who, in addition to mitral and/or aortic valve surgery, had De Vega's tricuspid annuloplasty. To determine the degree of tricuspid insufficiency (TI), right atrial pressure (PRA) tracings, biplane right ventricular cineangiograms, and ultrasonic Doppler flow patterns were obtained. The TI improved in 14 patients, but still remained mild to moderate in 20 patients. In addition, a mild-to-moderate tricuspid stenosis with pressure gradients from 2 to 7.8 mm Hg were found in 12 patients. There was no significant change in the mean PRA, with 8.0 +/- 4.5 mm Hg preoperatively and 7.5 +/- 3.5 mm Hg postoperatively at rest. During exercise, PRA rose to 17.0 +/- 6.5 mm Hg. The unsatisfactory results are also due to the persistent elevation of left atrial and pulmonary artery pressure after left heart surgery. De Vega's annuloplasty does not answer the tricuspid challenge, since the results are unpredictable.

Adolescent↗
PubMed1996

Is a bioprosthesis preferable in tricuspid valve replacement?

We reviewed clinical data on 29 patients (17 acquired disease and 12 congenital disease) who underwent tricuspid valve replacement using a Carpentier-Edwards porcine valve or a St. Jude Medical valve at Niigata University Hospital between January 1978 and October 1995, and analyzed risks related to the outcome. Age ranged from 8 to 62 years, and 17 females were included. New York Heart Association functional class was IV in 9 patients (31%). Previous valve surgery had been performed in 10 patients (34%). An urgent surgery was required in 4 patients and simultaneous mitral (with aortic) valve replacement in 13 (45%). Mean follow-up period was 6.7 years. Hospital mortality occurred in 5 patients (17.2%) and all of them had acquired valvular diseases with functional class IV. There were 6 late deaths and none of them related to the tricuspid prosthesis. Nonfatal morbidity occurred in 2 patients (a thrombosed valve and a chronic hemolytic anemia) with a St. Jude valve. Late functional class was I or II in all patients except 3 with severe left-ventricular dysfunction or bioprosthetic failure. Late postoperative echocardiographic examination showed moderate tricuspid stenosis or regurgitation in 3 patients with a Carpentier-Edwards valve. By the Kaplan-Meier method, functional class (P < 0.01) and baseline diseases (p < 0.05) were found to affect probability of freedom from cardiac- and/or valve-related mortality including hospital death. In conclusion, the present study indicates that similarly good outcome would be expected whether using a St. Jude Medical valve or a Carpentier-Edwards porcine valve, and that both functional class IV and combined acquired valvular disease are risk factors for mortality after tricuspid valve replacement. Patient-specific selection of the prosthesis would be preferable in tricuspid valve replacement.

Adolescent↗
PubMed1979

Morphological variations in pulmonary atresia with intact ventricular septum.

The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The degree of right heart hypoplasia was quantified by measuring right and left heart dimensions and comparing them with 20 normal infant hearts. Right ventricular cavity size was usually smaller than normal but constituted a spectrum ranging from tiny to a dilated ventricle larger than normal. There was a positive correlation between triscuspid annular size and right ventricular size but no correlation between the size of the pulmonary artery and the right ventricle. Successful pulmonary valvotomy with subsequent adequate right ventricular function would have been precluded by a tiny right ventricular cavity or infundibular atresia in 14 specimens and by severe tricuspid stenosis or regurgitation in an additional 4. Severe right or left ventricular endocardial fibroelastosis may have adversely affected ventricular function in several others. Ten specimens displayed convex bulging of the left ventricular septal surface. If these these anatomical findings are representative for the condition as a whole, they provide a good explanation for its disastrous prognosis.

Ebstein Anomaly↗
PubMed1994

Temporary tricuspid valve detachment in closure of congenital ventricular septal defect.

In a consecutive series of 149 patients with congenital ventricular septal defect (VSD), temporary tricuspid valve detachment was applied in 39 (detached group) to facilitate the transatrial approach for closure of the defect. Baseline characteristics showed that, preoperatively, the detached group were younger (1.3 +/- 2.3 vs. 3.5 +/- 4.1 years, P = 0.002), shorter (0.67 +/- 0.20 vs 0.87 +/- 0.34 m, P = 0.001), lighter (6.9 +/- 5.4 vs 13.5 +/- 12.0 kg, P < 0.002), and had a higher mean right atrial pressure (6 +/- 2 vs 4 +/- 3 mm Hg, P < 0.003), mean end-diastolic right ventricular pressure (10 +/- 3 vs 8 +/- 3 mm Hg, P < 0.01) and mean pulmonary vascular resistance (267 +/- 202 vs 170 +/- 131 dyn s cm-5, P < 0.02) on cardiac catheterization. At surgery the aortic cross-clamp time was longer (48 +/- 17 vs 39 +/- 15 min, P = 0.003). Seven patients died (2 detached, 5 not-detached), from causes not related to either tricuspid detachment or VSD closure. Follow-up was complete with a mean duration of 2.0 years (range 0.1-5.5). All 142 survivors were investigated by echocardiography, which showed normal tricuspid valve function in all but 29 patients who had trivial regurgitation (6 detached, 23 not-detached). There was no tricuspid stenosis. In 30 patients (8 detached, 22 not-detached) a trivial residual VSD could be detected. One reoperation (not-detached) was performed 12.5 months after the initial surgery for recurrent VSD.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2000

Replacement of the tricuspid valve in children with congenital cardiac malformations.

BACKGROUND AND AIM OF THE STUDY: When replacing the regurgitant tricuspid valve in children, the decision to use either a bioprosthesis or a mechanical valve remains controversial. METHODS: The atrioventricular valve for the pulmonary circulation was replaced in 11 young patients aged between 8 months and 13 years. Complications of congenital cardiac malformation were present in seven patients, and Ebstein's anomaly in three; tricuspid valvular regurgitation was an isolated lesion in one patient. A bioprosthesis was implanted on seven occasions, and a bileaflet mechanical valve on eight, including re-replacement of the valve in four patients. RESULTS: One patient died two years after implantation due to respiratory problems. Tricuspid stenosis due to valve calcification occurred in four bioprostheses at between four and nine years after initial replacement (57%). In three of these cases the native valve leaflets had not been removed. Thrombosis occurred in one patient with a mechanical valve; rereplacement was successful. Anticoagulant-related hemorrhage occurred in another patient. Among patients receiving a mechanical valve, 83% of valves were dysfunction-free after five and ten years. CONCLUSION: When replacing an atrioventricular valve for the pulmonary circulation in children, we prefer to use a low-profile mechanical valve, especially when extensive repair of intracardiac malformation has been carried out, but ventricular function is good. In children with poor cardiac performance, a bioprosthesis is preferred, with total resection of the native valve leaflets.

Adolescent↗
PubMed1988

Fontan repair for tricuspid atresia: experience with 50 consecutive patients.

From April, 1975, to October, 1985, 50 patients (mean age, 5.3 years; range, 2.5 to 14 years) underwent a Fontan repair for tricuspid atresia or severe tricuspid stenosis. Fifty-six palliative operations had been performed previously in 43 patients. Twenty-nine patients received an atriopulmonary connection by direct anastomosis [19], interposition of an aortic homograft [7], or a porcine heterograft [3]. An atrium-subpulmonary chamber connection was performed in 21 patients (Björk modification, 10; Dacron tube, 10; valved conduit, 1). Eleven patients (22%) died early, 6 of whom did not meet the established criteria for a Fontan operation. One patient died 6 months postoperatively of superior vena cava thrombosis. Four patients were reoperated on successfully. At a mean follow-up of 28.8 months, 28 patients are in New York Heart Association Functional Class I and 9 are in Class II; 1 patient was lost to follow-up. Thirteen patients underwent control catheterization fifteen days to four years postoperatively (median right atrial pressure, 14 mm Hg). Exercise testing in 9 asymptomatic patients, performed after a mean interval of 33 months, showed a moderate impairment of cardiac performance. We conclude that in select patients, a Fontan repair appears to be a reliable procedure at medium-term follow-up.

Adolescent↗
PubMed2003

Follow-up after surgical closure of congenital ventricular septal defect.

OBJECTIVES: The purpose of this retrospective study was to assess long-term outcome of children after surgical closure of a ventricular septal defect (VSD). MATERIAL AND METHODS: Between January 1992 and December 2001 a consecutive series of 188 patients (100 females) were operated for closure of a VSD. Temporary tricuspid valve detachment (TVD) was applied in 46 patients (24%) to enhance exposure of the defect using transatrial approach. Pre-operative baseline characteristics showed that the detached group was younger (0.79+/-1.8 vs 2.1+/-3.5 years, p=0.002) and had a lower weight (6.5+/-6.4 vs 10.0+/-11.0 kg, p=0.009). RESULTS: There was no difference in cross-clamp time (temporary TVD 36.2+/-11.3 vs non-temporary TVD 33.6+/-13.1 min, p=0.228). Postoperative echocardiography showed that 67 patients (36%) had trivial/minimal regurgitation, 10 patients (22%) from the temporary TVD group vs 57 patients (40%) from the non-detached group (p=0.02). There was no tricuspid stenosis. Hospital mortality comprised two patients (1%). One patient died due to a pulmonary hypertensive crisis and one in relation to an acute patch dehiscence for which an emergency reoperation was necessary. At first postoperative echocardiography no shunting was detected in 113 patients, trivial shunting in 73 and significant shunting in none. Multivariate logistic regression analysis revealed that weight at operation was a predictive factor for the occurrence of residual shunting (OR 0.95, C.I. 0.91-0.99). One patient with conduction disturbances needed a permanent DDD-pacemaker. Three patients were lost to follow-up. Mean follow-up time was 2.6 years (range 0.1-9.4). During follow-up no reoperations were necessary for closing a residual VSD. One patient died 7 months postoperative due to a bronchopneumonia. During follow-up in 37 (51%) of the 73 patients the trivial shunting disappeared spontaneously at a median time of 3.9 years. According to actuarial analysis all trivial shunting had disappeared at 8.4 years. CONCLUSION: Trivial residual shunting disappeared spontaneously at a median follow-up time of 3.9 years. During follow-up no patient needed to be reoperated for residual VSD. TVD proved to be a safe method to enhance the exposure of a VSD.

Adolescent↗
PubMed1993

Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients.

BACKGROUND: The carcinoid syndrome is a rare cause of acquired valvular heart disease. Although the typical echocardiographic features of carcinoid heart disease are well recognized, this large series provides new information about unusual manifestations of the disease as well as the role of Doppler echocardiography. METHODS AND RESULTS: Between 1980 and 1989, 132 patients with carcinoid syndrome underwent echocardiographic study. The echocardiographic, Doppler, and clinical features of the 74 patients (56%) with echocardiographic evidence of carcinoid heart disease are described. Among these patients, 97% had shortened, thickened tricuspid leaflets. Tricuspid regurgitation was present in all 69 patients with carcinoid heart disease who underwent Doppler examination, and it was of moderate or severe degree in 62 patients (90%). Severe tricuspid regurgitation was characterized by a dagger-shaped Doppler spectral profile with an early peak pressure and rapid decline. The pressure half-time was prolonged (mean, 116 msec), which is consistent with associated tricuspid stenosis. The pulmonary valve appeared thickened, retracted, and immobile in 36 patients (49%) and was diminutive to the extent of not being visualized in an additional 29 patients (39%). Among the 47 patients who underwent Doppler evaluation of the pulmonary valve, regurgitation was present in 81%, and stenosis was present in 53%. Left-sided valvular involvement was present in five patients (7%), four of whom had patent foramen ovale or carcinoid tumor involving the lung. Previously undescribed myocardial metastases were present in three patients (4%) and were confirmed by biopsy in each case. Small pericardial effusions were present in 10 patients (14%). Patients with and without echocardiographic evidence of carcinoid heart disease did not differ with regard to sex, age, location of the primary tumor, duration of diagnosis, or duration of symptoms of carcinoid syndrome. However, the mean pretreatment level of urinary 5-hydroxyindoleacetic acid was higher in patients with carcinoid heart disease than in patients without carcinoid heart disease (270 versus 131 mg/24 hrs, p < 0.001). The symptom of dyspnea was more prevalent among patients with carcinoid heart disease than in patients without the disease (54% versus 27%, p = 0.003); as expected, heart murmurs were also noted more frequently in patients with disease (92% versus 43%, p < 0.0001). Treatment regimens and response to therapy were similar in the two groups. Survival of patients with echocardiographic evidence of carcinoid heart disease was reduced compared with those without cardiac involvement (p = 0.0003). ECG and chest roentgenographic findings in patients with carcinoid heart disease were nonspecific. CONCLUSIONS: The broad spectrum of carcinoid heart disease is detailed in this large series. This includes not only right-sided valvular lesions but also left-sided involvement, pericardial effusion, and myocardial metastases.

Carcinoid Heart Disease↗
PubMed1982

Raghib's syndrome: a report of two cases.

We treated two patients with a rare developmental complex. The persistent left superior vena cava draining into the left atrium (PLSVC into LA) was associated with an absent coronary sinus and an atrial septal defect. Ligation of PLSVC and patch-repair of the atrial septal defect were successfully performed in one stage. The atrial septal defect was located in the upper and posterior aspect of the interatrial septum and appeared to be an unique type of atrial septal defect. In the other patient, additional multiple cardiac defects were associated with this syndrome, including ventricular septal defect, pulmonary stenosis, tricuspid insufficiency, and complete transposition of the great arteries. Palliative Blalock procedure was used for this patient. The PLSVC into LA was discovered accidentally in both cases during heart catheterization and it was clearly demonstrated by venography. For a preoperative recognition of PLSVC, computed tomograms of the heart are of great assistance. Surgical correction of the persistent superior vena cava was emphasized for treatment of this syndrome.

Adult↗
PubMed2003

Congenital heart disease with hypereosinophilic syndrome.

The idiopathic hypereosinophilic syndrome, common in adults, is a leukoproliferative disorder marked by a predilection to damage specific organs. This report describes a unique case of an infant with tricuspid stenosis, a hypoplastic right ventricle, severe pulmonary stenosis, patent ductus arteriosus, and a patent foramen ovale associated with hypereosinophilic syndrome. Although treatment with high-dose oral steroids failed to decrease the leukocyte counts, a right classic Blalock-Taussig shunt through a right thoracotomy resolved her cyanosis.

Female↗
PubMed2004

Phenotypic variability in Hey2 -/- mice and absence of HEY2 mutations in patients with congenital heart defects or Alagille syndrome.

The genetic alterations leading to congenital heart defects (CHD) are still poorly understood. We and others have recently shown that in mice loss of Hey2 results in a high incidence of fatal ventricular and atrial septal defects, combined with tricuspid stenosis or atresia in some cases. The phenotype has been postulated to resemble human tetralogy of Fallot. Our analysis of CD1 outbred mice suggests that phenotypic consequences of Hey2 loss can be quite variable and dependent on modifier genes as we detected only isolated VSDs with lower prevalence and a significantly reduced mortality rate in this strain. Since Hey2 is one of the few Notch target genes, it is also conceivable that HEY2 mutations may account for cases of Alagille syndrome (AGS: variable combinations of heart, skeleton, eye, and facial malformations and cholestasis), in which the typical mutations of the Notch ligand JAG1 cannot be found. To clarify the role of HEY2 in human CHD and AGS, we screened by direct sequencing 23 children with CHD and 38 patients diagnosed with AGS, which lack mutations in the JAG1 gene. We found two types of silent changes in the coding region: a CTT-->CTG transition in exon 3 and a CTG-->CTC polymorphism in exon 5. Furthermore, a heterozygous SNP in the splice donor site of exon 4 was detected that is unlikely to disrupt splicing. Although the high incidence and variability of human congenital heart defects implies a multifactorial genetic basis, our results suggest that mutation of HEY2 is not a major contributing factor.

Adolescent↗
PubMed2000

Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is a lethal condition without intervention. Cross-sectional echocardiography is the diagnostic investigation of choice. Intravenous infusion of prostaglandin is employed to maintain ductal patency and allow mixing of blood, thus improving tissue oxygenation. Balloon atrial septostomy is recommended once the diagnosis is made. The arterial switch is accepted as the best option for simple TGA. Late follow-up includes survivors of the intra-atrial repair (Mustard and Senning operations), and the emerging cohort of survivors of the arterial switch procedure. Arrhythmia, baffle stenosis, tricuspid valve dysfunction, systemic ventricular dysfunction, and sudden death may occur late during follow-up after the Mustard or Senning procedure. There are less data for late follow-up after arterial switch; however, late death is rare, usually is related to reoperation, and important arrhythmias are uncommon. The long-term fate of the coronary circulation is unknown but coronary arterial obstruction has been reported. Continuing long-term surveillance is essential to detect the development of late problems in all groups of survivors.

Journal Article↗
PubMed1976

Clinical study on the flow murmurs at the defect area of atrial septal defect by means of intracardiac phonocardiography.

In order to study flow murmurs through atrial septal defects, right heart catheterization was performed on 48 patients of secundum type, four of primum type, and five of probe-patent foramen ovale, with the double-lumen phonocatheter of Lewis, at the tip of which barium titanate was mounted. The flow murmurs at the defect area were classified into three patterns: v murmur, atriosystolic murmur, and mid-diastolic murmur. V murmur was continuous, extending from late systole to diastole, of low to medium pitch, closely related to atrial v wave and augmenting with expiration. It had no significant correlation to the ratio of left-to-right shunt. It was recorded in 32 out of 48 cases of secundum type and one of primum type, but not observed in probe-patent foramen ovale. Atriosystolic murmur was noted in 17 of 48 cases of secundum type and one of primum type. It was connected with atrial a wave. Mid-diastolic murmur was found at the defect area in four subjects of secundum type. It was thought to be an independent entity from v murmur and to be another one due to shunt flow through the septal defect, since it had no relation to v wave but it was localized between v and a waves in the pressure curve of the right atrium. It is different in localization from mid-diastolic murmur due to relative tricuspid stenosis at the inflow tract of right ventricle.

Adult↗
PubMed1997

[Primary cardiac lymphoma: presentation of a case and review of the literature].

We describe a case of primary cardiac lymphoma presenting with chest pain, complete AV block, negative T waves, and infiltration of the basal segments and right free ventricular wall on echocardiography, interpreted initially as hypertrophy. One month later the patient was readmitted with systemic disease and cardiac insufficiency. Furthermore multicentric myocardial infiltration with a nodular mass in the right atrium producing severe tricuspid stenosis was demonstrated. Surgical biopsy was performed and a high grade non-Hodgkin's lymphoma diagnosed. The patient died during the immediate post-operative period without receiving specific chemotherapeutic treatment. Reviewing the published cases, we found that primary cardiac lymphomas are fast growing tumors that infiltrate predominantly the right cavities and have limited therapeutic options.

Fatal Outcome↗
PubMed1981

Clinical presentation and non-invasive diagnosis of right heart masses.

Right-sided cardiac masses do not have a uniform clinical presentation. Successful diagnosis is dependent on a high index of suspicion and appropriate echocardiographic examination. Five cases of right sided intracardiac masses have been detected during the period that echocardiography has been routinely available to us--two of right atrial myxoma, one of right atrial thrombus, and two of right ventricular tumour. M-mode echocardiography identified four of the five cases. Two-dimensional echocardiography was necessary to establish the non-invasive diagnosis in the fifth case. Echocardiography should be used in any patient in whom a right sided mass is suspected, or in those patients presenting with signs of tricuspid regurgitation, tricuspid stenosis, pulmonary stenosis, cyanotic heart disease, progressive right heart failure, constrictive pericarditis, or pulmonary emboli without obvious source.

Adult↗