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Surgical treatment of Ebstein's anomaly. Early and late results in seven consecutive cases.

The surgical treatment of Ebstein's anomaly is discussed on the basis of our experience with 7 patients who underwent operation at this clinic during the last ten years. The age of the patients (2 males, 5 females) ranged from 17 to 47 years (mean 27 years) at the time of operation. Two patients were in functional class II and five in classes III or IV (N.Y.H.A.). The Wolff-Parkinson-White syndrome was concomitant in 2/7 cases and patent foramen ovale or atrial septal defect (ASD) with right-to-left shunt was present in 6/7 cases. The valvular anomaly was typical in 5 patients and stenosis of displaced tricuspid leaflets into right ventricular outflow region was detected in two patients. Tricuspid valve replacement (TVR) with disc valve prosthesis was performed in 5 patients (Björk-Shiley model in 4 cases, Kay-Shiley model in one) and tricuspid valve commissurotomy in two patients. All six patients with interatrial communication underwent closure of this communication simultaneously. One patient had concomitant epicardial mapping and attempted division of the anomalous conduction pathways was unsuccessful. This patient died on the 14th postoperative day from ventricular fibrillation. No other early or late mortality was recorded. Life-long anticoagulation therapy was introduced in all 5 patients with TVR. Thrombotic malfunction of the prosthetic valves necessitated re-operation and new prosthetic valve replacement in two patients 15 and 16 months after the first TVR. One of these patients had recurrent prosthetic thrombosis twice which was successfully treated with streptokinase. The other patient's prosthesis is functioning well 18 months after re-operation. All 6 long-term survivors were improved and remained in good condition, in functional class I, 1, 3, 3, 8, 9, and 10 years, respectively, after operation.

Adolescent↗

Unusual complications of endocardial pacing.

This report concerns two unusual complications of endocardial pacing: (1) simultaneous thrombosis of the superior and inferior venae cavae and (2) supravalvular, valvular, and infravalvular stenosis of the tricuspid valve. The management of both cases required removal of all endocardial leads with the aid of cardiopulmonary bypass, with inferior vena cava thrombectomy in the first case and tricuspid valve replacement in the second. From our review of the literature, we have reached the following conclusions: (1) Clinical thrombosis secondary to endocardial pacing leads is rare (1.2%), but venography in asymptomatic patients revealed venous thrombosis in 44% of the patients studied. (2) Endocardial pacing leads should have the smallest outer diameter possible, preferably with an electro-negative, thromboresistant surface, and should be implanted with minimal redundancy. The risk of venous thrombosis should be kept in mind when recommending atrioventricular sequential pacing systems requiring multiple leads passing through the superior vena cava. (3) Retained leads which are infected or have migrated and become redundant or looped are at risk of causing septicemia, thrombosis, or septic embolization and should be removed. The high incidence of subclinical venous thrombosis suggests that reasonable efforts be made to remove all retained leads.

Aged↗

[A case report of complex cardiac malformation treated with total cavopulmonary connection after pulmonary artery banding].

A two-year-old boy with cardiac malformation including hypoplastic right ventricle, severe stenosis of the tricuspid valve, atrial primum defect and ventricular septal defect underwent total cavopulmonary connection after pulmonary artery banding, which was done to tract cardial failure at six months after birth. Under cardiopulmonary bypass, an intraatrial cavocaval channel was constructed with a prosthetic patch of expanded polytetrafluoroethylene (EPTFE). The superior vena cava was transected and each end of the superior vena cava was anastomosed to the pulmonary artery in an end-to-side fashion. Pulmonary artery pressure registered 15 mmHg on cardiac catheterization after operation, and angiography showed smooth blood flow to the pulmonary artery from the vena cava. We suggest that a Fontan-type operation could be indicated for the patients younger than 4-year-old having acceptable pulmonary resistance if the symptoms of hemoconcentration and hypoxia are life-threatening.

Anastomosis, Surgical↗

Surgical alternatives to the Fontan procedure incorporating a hypoplastic right ventricle.

BACKGROUND: Frequently the definitive operation for patients with a right ventricle (RV) that is too small to support full cardiac output is a modified Fontan operation. However, other surgical options exist that incorporate a small RV in the atriopulmonary pathway when biventricular repair is not feasible because of RV or tricuspid valve hypoplasia. The risks and benefits of these options have not been well defined. METHODS AND RESULTS: Between 1988 and 1993, 8 patients (6 with pulmonary atresia and intact ventricular septum and 2 with tricuspid valve stenosis and RV hypoplasia) underwent a cavopulmonary connection, which allowed right atrial blood to flow either to the pulmonary artery via the RV or directly via the cavopulmonary anastomosis. Age at surgery ranged from 1.5 to 9 years. The proximal right pulmonary artery was ligated in 5 patients, and the atrial septal defect was closed during the same procedure in 7 of the 8 patients. The echocardiographic right ventricular-left ventricular volume ratio ranged from 9% to 25%, and tricuspid valve z-scores ranged from 0 to -4. There were no deaths at a median follow-up of 24 months (range, 7 to 61 months). Mild exertional limitation was evident in only one patient. Postoperative echocardiograms demonstrated pulsatile systolic flow across the RV outflow tract in 5 patients and low-velocity diastolic-systolic flow in a sixth patient with extreme tricuspid valve hypoplasia. At postoperative cardiac catheterization (6 patients) right atrial mean pressures ranged from 7 to 13 mm Hg and mixed venous saturations from 62% to 70%. CONCLUSIONS: Right atrial decompression via a superior vena cava-to-pulmonary artery anastomosis allows incorporation of a small RV into the pulmonary circulation and closure of the atrial septum, with excellent results to date.

Cardiac Catheterization↗

Extracardiac right atrium-to-right ventricle homograft for uncorrectable tricuspid valve disease.

Surgically uncorrectable tricuspid valve disease in children is rare. However, when it happens the surgical options are very limited. Tricuspid valve replacement using a mechanical valve or stented bioprosthesis is impractical. Use of homografts in the "anatomic position" has its limitations. We report here the use of an extracardiac homograft connection between the right atrium and right ventricle in a 16-month-old boy in whom severe tricuspid valve stenosis developed after surgical repair of a complex ventricular septal defect associated with dextrocardia and anomalous systemic venous drainage. The patient remains well receiving no cardiac medication 12 months after the procedure.

Aorta↗

[A case of tricuspid and mitral valve replacement for incomplete endocardial cushion defect].

A 56-year-old female who was diagnosed incomplete endocardial cushion defect, underwent closure of ostium primum defect and repair of mitral cleft 2 years ago. Mitral and tricuspid ring annuloplasty was done 9 months after the first operation because of an increment of mitral and tricuspid valve regurgitation. The Third operation, mitral valve replacement by mechanical valve and tricuspid valve replacement by bioprosthetic valve, was performed because of the gradually increased mitral stenosis and tricuspid regurgitation. Post-operative course was uneventful. Resected anterior cusp of mitral and tricuspid valve revealed hypertrophy and shortness macroscopically, and revealed fibrosis and calcification histopathologically. It was considered that remarkable regurgitation was due to secondary change of both valves. We concluded that valve replacement should be performed for case of secondary change of atrioventricular valve in adult patient.

Endocardial Cushion Defects↗

Noninvasive graphic evaluation: phonocardiography and echocardiography.

Noninvasive graphic techniques are an invaluable tool in the diagnosis, management, and followup of patients with valvular heart disease. They are both sensitive and specific and therefore often eliminate the need for more invasive and more expensive diagnostic procedures. When cardiac catheterization and/or cineangiography are necessary to obtain definitive hemodynamic or anatomic information, prior noninvasive graphic evaluation frequently directs the cardiologist to the appropriate type of study to be performed. This is especially true of subtle tricuspid valvular disease, unsuspected silent stenotic valvular lesions, or unusual causes of aortic regurgitation. Because these studies are noninvasive and can be repeated as often as necessary, they are ideal techniques for the longitudinal followup of the patient. Additionally, these techniques are excellent teaching tools, and they allow cardiologists to continually check and refine their clinical skills, which are the foundation of the bedside evaluation of the patient with valvular heart disease.

Aortic Valve Insufficiency↗

Carcinoid heart disease: diagnosis by two-dimensional echocardiography.

We prospectively studied 14 patients with the carcinoid syndrome to determine if two-dimensional echocardiography could detect the nature and extent of valvular abnormalities. Eight of the 14 patients had definite abnormalities of the right-sided cardiac valves. The tricuspid valve had a characteristic appearance, similar to previously described pathologic findings. The leaflets appeared diffusely thickened, shortened and stiff without evidence of commissural fusion. Saline contrast studies demonstrated tricuspid regurgitation, which corresponded to the severity of the tricuspid valve involvement. The pulmonary valve could only be adequately assessed in seven of 14 patients, and morphologic abnormalities similar to those in the tricuspid valve were found. Follow-up studies have shown progression of cardiac disease in six of eight patients. We conclude that two-dimensional echocardiography can detect the characteristic cardiac abnormalities in the carcinoid syndrome and may be a useful tool for following their progression.

Adult↗

Accumulation of T lymphocytes and expression of interleukin-2 receptors in nonrheumatic stenotic aortic valves.

OBJECTIVES: Cell-specific antibodies were used to identify immunocompetent cells in a comparison of valves from patients who had symptomatic tricuspid aortic stenosis with subjects who had no evidence of valvular heart disease. BACKGROUND: Nonrheumatic valvular aortic stenosis is the most common valvular heart disease among adults. The biologic processes involved in the development of this disease are poorly understood. METHODS: Tricuspid stenotic aortic valves were obtained from 19 patients undergoing surgery for nonrheumatic valvular aortic stenosis, and 10 control valves were collected at autopsy. The valves were fixed in formaldehyde, cryosectioned and stained with antibodies against fibroblasts, endothelial cells, macrophages, T lymphocytes and interleukin-2 receptors. A subset of valves were also analyzed with antibodies against T-helper cells and cytotoxic T cells. RESULTS: Stenotic valves were characterized by a basal accumulation of calcium deposits and a cell-rich subendothelial thickening. The immunohistologic analysis indicated that the cells in the subendothelial connective tissue were fibroblasts. T lymphocytes appeared to be the most common cell type in the vicinity of the calcium deposits and were also found close to the endothelial lining of the valves. T-helper cells were more frequent than cytotoxic T cells. Expression of interleukin-2 receptors occurred at the same location as T lymphocytes. Control valves lacked subendothelial thickening and contained only few cells reacting with antibodies against lymphocytes and macrophages. CONCLUSIONS: The presence of activated T lymphocytes in tricuspid stenotic valves suggests that immunologic mechanisms may be involved in the etiology of nonrheumatic aortic stenosis.

Aged↗

Cyanotic congenital heart disease with decreased pulmonary blood flow in children.

Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.

Algorithms↗

[Congenital abnormalities of the tricuspid valve and Ebstein's anomaly with pulmonary valvular stenosis].

We are reporting a case of Ebstein's anomaly of the tricuspid valve and another case of a congenital double tricuspid lesion, both associated with pulmonary valve stenosis and with similar hemodynamics and physiopathology. Given that there are few reported cases in the literature, we analyzed the clinical and electrocardiographic findings of both. The utility of the echocardiogram is discussed in the diagnosis of such abnormalities and the angiographic and hemodynamic data commented upon. It is emphasized that right ventricular hypertension (105 mm Hg, and 98 mmHg, respectively) modifies the cardiopathic hemodynamics and its natural history, that was favorably modified by isolated corrective surgery of the pulmonary obstruction. The postoperatory transvalvular gradients were measured at 27 and 11 mm Hg, respectively. After having revised the pertinent bibliographic literature, we commented upon the factors that give such a high mortality rate in Ebstein's surgery and the congenital abnormalities which are most frequently associated.

Child↗