Successful implantation of leadless pacemaker in a patient with giant right atrium and tricuspid valve stenosis.

With the steadily increasing amount of leadless pacemaker implantations performed worldwide, it has called attention to the delivery difficulty in patients with severe large right heart. Nevertheless, limited studies have reported leadless pacemaker implantation in patients with tricuspid stenosis. Here, we report the successful implantation of leadless pacemaker in a 60-year-old female patient with giant right atrium and tricuspid valve stenosis. It is highlighted that leadless pacemaker should not be discouraged even if there are tricuspid valve stenosis and giant right atrium.

Valvular heart disease in Antiphospholipid antibody syndrome: Isolated Tricuspid stenosis.

Antiphospholipid antibody syndrome (APLS) is a rare disorder characterized by a hypercoagulable state. Manifestations include arterial or venous thrombosis, recurrent fetal wastage, coronary artery disease, valvular heart disease, dilated cardiomyopathy, pulmonary artery hypertension, and intracardiac thrombus. Most commonly mitral valve is affected followed by aortic and then tricuspid valve. In this report, a rare case of spontaneous aortic thrombosis with tricuspid stenosis uncomplicated by other valve lesions is presented with clinical and echocardiographic studies and computed tomographic images.

Sudden death due to nonarrhythmic cause in a patient with L-TGA.

We report a case of sudden death in a clinically stable adult with l-transposition of the great arteries (l-TGA). Sudden death has been reported to be the leading cause of death in l-TGA and is often attributed to arrhythmias in the absence of another identifiable cause. However, the contribution of nonarrhythmic causes to the burden of sudden death in this population is unknown. Comprehensive postmortem investigation, including autopsy and pacemaker interrogation, demonstrated that the cause of death was massive pulmonary hemorrhage due to stenosis of the patient's mechanical tricuspid (systemic AV) valve. This report highlights the important contribution of nonarrhythmic causes of sudden death in this population and the value of autopsy and device interrogation in determining true cause of death.

Spinal surgery in the univentricular heart--is it viable?

INTRODUCTION: The management of patients with Fontan physiology who undergo scoliosis surgery is difficult. The purpose of this article was to describe our experience in the management of patients with Fontan circulation undergoing spinal surgery for correction of scoliosis. MATERIALS AND METHODS: This was a retrospective study including patients with Fontan physiology who underwent spinal orthopaedic surgery. Anaesthetic management, post-operative complications, paediatric intensive care unit and total hospital stay, and the need for blood transfusions were analysed. RESULTS: We identified eight children with Fontan physiology who had undergone spinal surgery from 2000 to 2010. All patients were receiving cardiac medications at the time of spinal surgery. The mean age at surgery was 14.8 years (range 12-21). In all, three patients needed inotropic support with dopamine (3, 5, and 8 µg/kg/min), which was started during surgery. During the immediate post-operative period, one patient died because of hypovolaemic shock caused by massive bleeding and dysrythmia. Mean blood loss during the post-operative period was 22.2 cc/kg (7.8-44.6). Surgical drainages were maintained for a mean time of 3 days (range 1-7). The mean hospital stay was 9.2 days (range 6-19). Pleural effusions developed in two patients. On follow-up, one patient presented with thoracic pseudarthrosis and another with a serohaematoma of the surgical wound. CONCLUSIONS: Spinal surgery in patients with Fontan circulation is a high-risk operation. These patients must be managed by a specialised team.

Percutaneous transvenous tricuspid commissurotomy in a patient with congenitally corrected transposition of great arteries: a case report.

Percutaneous balloon tricuspid valvotomy was successfully performed in a 45-year-old female with congenitally corrected transposition of great arteries with severe rheumatic left atrioventricular (tricuspid) valve stenosis. Technical modifications in the standard procedure were made keeping in mind the left-handed ventricular loop, left and anterior aorta, wedged pulmonary valve between the interatrial septum and the mitral valve with deviation of the atrial septum away from the ventricular septum, side-by-side positioned ventricles with an added superoinferior obliquity produced by excessive tilting, and an abnormal orientation of ventricular mass in relation to the thorax, with the apex pointing slightly rightwards. A final valve area of 1.4 cm(2) and a fall in the peak/mean left atrial pressures from 37/32 mm Hg to 13/10 mm Hg were achieved without complications. This case of percutaneous transvenous commissurotomy is unique in view of the rarity of the combination of this congenital heart disease and rheumatic heart disease and successful commissurotomy in such an unusual cardiac anatomy using the Inoue technique.

Case of calcific tricuspid and pulmonary valve stenosis.

Isolated right-sided valvular disease is a much less recognised entity when compared with left-sided valvular heart disease. Almost all the cases of combined pulmonary valve with tricuspid valve involvement are a consequence of underlying carcinoid heart disease. Moreover, severe calcification of tricuspid valve is an extremely unusual finding. We report a case of a severe calcific tricuspid valve stenosis along with severe pulmonary valve stenosis where the exact aetiology could not be established. On reviewing the literature, we did not find any reports describing such a morphology.

Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease.

Carcinoid disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells. It is the most common tumour affecting the small bowel. The majority of patients who progress to carcinoid syndrome develop cardiac disease selectively involving the right side of the heart, whereas left heart disease is unusual. The most common cause of death is dilatation and dysfunction of the right ventricle. Right ventricular dysfunction is largely secondary to pathological endocardial fibrosis of the tricuspid and pulmonary valves, presenting with regurgitation and stenosis. Average survival falls to only 11 months with the onset of symptoms, but recent evidence suggests that survival can be improved by early surgery in selected individuals. This article reviews the particular role that cardiovascular magnetic resonance imaging has in the management of carcinoid heart disease.

Double Valve-in-Valve Transcatheter Valve Replacements for Failed Surgical Bioprosthetic Aortic and Tricuspid Valves.

Transcatheter technology has revolutionized the treatment of valvular disease in the field of cardiology and cardiac surgery. We present an interesting case of a patient with prior double valve replacements, which had degenerated after a decade, with symptoms of decompensated heart failure. The patient was successfully treated with double valve-in-valve transcatheter aortic and tricuspid valve replacement.

Respirophasic desaturation in multivalvar rheumatic heart disease.

A 28-year-old lady with rheumatic heart disease presented with dyspnoea and overt right heart failure and was admitted for stabilization. She had respirophasic desaturation predominantly during inspiration. Trans-oesophageal echocardiography revealed the aetiology of the same and dictated the management strategy.

A rare case: bioprosthetic tricuspid valvuloplasty dramatically improved hepatic encephalopathy.

A 51-year-old woman was diagnosed as severe stenosed tricuspid bioprosthetic valve. She had developed an encephalopathy due to elevated serum ammonia concentration caused by congestive hepatic failure. Re-tricuspid valve replacement was deemed too risky, and balloon bioprosthetic valvuloplasty was instead planned. This procedure was successfully performed using a standard mitral valvuloplasty protocol. The 30-mm INOUE-BALLOON was inflated five times. The mean pressure gradient across the bioprosthetic valve decreased from 7.8 to 3.5 mmHg, and the tricuspid valve orifice area increased from 1.09 to 3.13 cm(2), without worsening of the tricuspid valve regurgitation. Finally, her hepatic encephalopathy was dramatically improved.

[Unusual cyanosing heart defect due to supravalvular tricuspid obstruction in an infant]. / Una forma rara de cardiopatía cianosante por obstrucción supravalvular tricuspídea en el lactante.

We present a case involving a rare form of cyanotic congenital heart disease in a 2-month-old neonate. The initial diagnosis was thought to be tricuspid dysplasia with right-to-left shunting through an interatrial communication. However, surgery showed the presence of a pedunculated mass that prolapsed into the tricuspid valve orifice, thereby causing severe obstruction to right ventricular filling. This mass was attached to the lower part of the atrial septum. Pathological examination showed fibrotic myxoid tissue. After the mass was resected surgically, the tricuspid valve appeared normal. We comment on the pathogenesis and the differential diagnosis of this rare entity.

Bioprosthetic dysfunction caused by preserved native valve in tricuspid position.

We report a 4-year-old girl who underwent replacement of a bioprosthetic valve in the tricuspid position due to rare bioprosthetic dysfunction. The bioprosthetic valve showed marked tricuspid insufficiency as well as stenosis caused by the adhesion of the preserved native valve leaflets to the undersurface of the implanted bioprosthesis.

Percutaneous transcatheter balloon valvuloplasty for bioprosthetic tricuspid valve stenosis in a patient with orthotopic heart transplant.

A 47-year-old man with history of familial cardiomyopathy and orthotopic heart transplant at the age of 34 years presented severe tricuspid stenosis (TS) with a mean transvalvular gradient of 10 mm Hg. The patient opted against tricuspid valve replacement and was offered percutaneous transcatheter tricuspid balloon valvuloplasty (PTTBV). To the best of our knowledge, this is the first report of a PTTBV in an orthotopic allograft, and demonstrates excellent clinical outcome at 1 year.

Thrombotic Obstruction of a Melody Valve-in-Valve Used for Prosthetic Tricuspid Stenosis.

The percutaneous Melody valve is occasionally used for the treatment of bioprosthetic valve dysfunction and is an attractive option for patients at high risk for repeat operation. Anticoagulation is not routinely recommended following Melody valve placement. We present the case of a young woman who developed subacute spontaneous thrombosis of her Melody valve 15 months after placement. Her clinical status was compromised by severe tricuspid prosthesis stenosis and right-to-left shunting across a small residual atrial septal defect. Surgical replacement was required. To our knowledge, this is the first reported case of spontaneous thrombosis of a Melody valve in the tricuspid position.

Combined tricuspid and pulmonic stenosis. Clinical, echocardiographic, hemodynamic, surgical, and pathological features.

A 30-year-old man with a history of a murmur since childhood had progressive ascites for 2 years. The patient was found to have severe stenoses of the pulmonic and tricuspid valves with a well-developed right ventricle. Impressive clinical improvement occurred after pulmonic valvotomy, infundibulectomy, and replacement of the stenotic tricuspid valve with a porcine xenograft. The clinical, echocardiographic, hemodynamic, surgical, and pathological features are presented.

Congenital tricuspid stenosis treated by a palliative open operation. Report of a case.

The case is presented of a patient with congenital tricuspid stenosis who was treated successfully by a palliative open operation. The preoperative diagnosis was tricuspid atresia. At operation, however, congenital tricuspid stenosis was detected and managed by commissurotomy.. The postoperative course was very good. Later, the associated ventricular septal defect (VSD), pulmonary annular stenosis, and probably the atrial septal defect (ASD) will be corrected. We emphasize the importance of diagnosing congenital tricuspid stenosis correctly, because of the possibility of operating upon the valve itself.

Echocardiographic diagnosis of atrial septal aneurysm in an infant with hypoplastic right heart syndrome.

Echocardiographic findings are described in a patient with hypoplastic right heart syndrome (pulmonary atresia type with intact ventricular septum and small right ventricular cavity) who had an associated atrial septal aneurysm. An unusual appearance of echoes behind the aorta bulging into the left atrium in diastole on both the M-mode and cross-sectional echo suggested this diagnosis prior to cardiac catheterization. The angiographic findings confirmed the diagnosis of right ventricular hypoplasia, pulmonary atresia and the large atrial septal aneurysm. The infant died after surgery and the atrial septal aneurysm was observed at autopsy. The importance of the diagnosis of the atrial septal aneurysm and its association with restriction of right-to-left atrial shunting prompts this report.

Acute and early experimental tricuspid valvular stenosis in dogs.

Acute and early (two months) experimental tricuspid valve stenosis was produced in 20 dogs. Clinical and hemodynamic alterations that resulted from severe anatomic tricuspid valve narrowing were surprisingly mild. In the acute stenosis studies, the normal tricuspid valve area of 8.2 +/- 0.3 sq cm was narrowed to less than 1.0 +/- 0.1 sq cm with a resulting right auricle-right ventricle diastolic gradient of 3.7 +/- 0.7 mm Hg. In early studies, a decrease in tricuspid valve area from 7.3 sq cm to 1.6 +/- 0.3 sq cm produced a diastolic gradient of 1.8 +/- o.2 mm Hg. After 60 days, overt signs of right-sided failure (pleural effusions and ascites) were absent, and histological evidence of passive congestion (liver and spleen) was unpredictable. The systemic and portal venous vascular beds appear to act as excellent buffers against increases in right-sided cardiac pressures. We conclude that isolated narrowing of the tricuspid valve must be very severe to cause notable clinical and hemodynamic changes.