Case of calcific tricuspid and pulmonary valve stenosis

Abstract

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.

Background

Multivalvular heart disease is seen in various pathologies, namely rheumatic heart disease, infective endocarditis, mediastinal radiation therapy and connective tissue disorders.¹ Degenerative heart disease with coronary artery disease are other causes that present with aortic valve involvement and mitral regurgitation.² Most of these causes however include combination of left-sided valves, either alone or along with ‘functional’ tricuspid regurgitation secondary to pulmonary hypertension, right ventricular dilatation or dysfunction.

Isolated right-sided multivalvular involvement is extremely uncommon. The most likely cause includes malignant carcinoid syndrome where 20% of the patients may present with cardiac involvement at the initial presentation.³ Other causes could be infective endocarditis, rarely congenital heart diseases of ‘hypoplastic right heart syndrome’⁴ and Ebsteins anomaly.⁵

We describe a case of middle-aged woman with no significant medical history presenting with severe calcific tricuspid valve stenosis with severe pulmonary valve stenosis.

We report this case to highlight the rarity of the disease along with uncertainty of the underlying cause which could not be ascertained even after investigations.

Case presentation

A 45-year-old woman presented to cardiac outpatient department with dyspnoea on exertion New York Health Association classification II with generalised fatigue for the past 2 years. She also reported occasional substernal chest pain. There was no history suggestive of rheumatic fever, infective endocarditis, drug intake or knowledge of any childhood murmur in the past. On examination the patient had elevated jugular venous pressure with prominent ‘a’ wave. The patient had hepatomegaly, pedal oedema with minimal ascites. Her first heart sound was soft, with a diastolic rumbling murmur in third and fourth intercostal spaces at the left sternal border. A harsh ejection systolic murmur was audible in the second intercostal space on the left side. Her ECG revealed right bundle branch block with right axis deviation, right atrial enlargement and right ventricular hypertrophy (figure 1). On 2D echocardiography, right-sided chambers were dilated with severely calcified tricuspid valve (figure 2). All the leaflets of the valve were heavily calcified with maximum gradient of 35 mm Hg and mean gradient of 14 mm Hg at the heart rate of 94 beats per minute (figure 3). There was no tricuspid regurgitation. Pulmonary valve was thickened, with severe valvular and infundibular stenosis with peak gradient 135 mm Hg (figure 4). Right atrium and right ventricle were dilated with leftward shifting of interatrial septum suggestive of elevated mean right atrial pressures; however, right ventricular function was preserved. The left-sided valves were normal with ejection fraction of 60%.

Figure 1.

ECG depicting right bundle branch block, right atrial enlargement with right ventricular hypertrophy.

Figure 2.

Transthoracic echocardiography: still colour Doppler image showing thickened and calcified tricuspid valve with dilated right-sided chambers in apical four-chamber view. Both anterior tricuspid leaflet and septal tricuspid leaflet are thickened with high velocity jet seen across the valve.

Figure 3.

Transthoracic echocardiography: right ventricular inflow view showing thickened anterior tricuspid leaflet and posterior tricuspid leaflet.

Figure 4.

Transthoracic echocardiography: high parasternal short axis view showing thickened pulmonary valve with continuous wave Doppler gradient of 135 mm Hg. Presence of ‘dagger’-shaped late-peaking curve denotes additional infundibular stenosis.

The initial possibility that was considered was carcinoid heart disease and the patient was extensively worked up for the same. Symptoms pertaining to carcinoid syndrome of facial flushing, diarrhoea or bronchospasm were not present in the patient. Though the presentation of patients with pure right-sided heart failure without symptoms of carcinoid tumour is rare, cases have been reported.⁶ On evaluation the two biochemical markers serum chromogranin A levels and 24-hour urine 5-hydroxyindoleacetic acid were normal. Whole body positron emission tomography was then conducted which showed no increased metabolic activity. Other serum markers including antinuclear antibody levels were also normal.

Coronary angiogram depicted normal coronary arteries and a ring of dense calcification in the position of the tricuspid valve was seen on fluoroscopy (figure 5). Cardiac MRI was performed which dilated right-sided chambers along with T2 hypointensity in the region of tricuspid valve suggestive of intense calcification (figure 6).

Figure 5.

Fluoroscopic image depicting ring calcification of the tricuspid valve (black arrow).

Figure 6.

Cardiac MRI revealing T2 hypointensity in the region of tricuspid valve suggestive of calcification (red arrow).

Outcome and follow-up

The patient was thereafter managed on low-dose diuretics on which symptoms improved. The patient was counselled regarding the need for double valve repair or replacement; however, due to financial constraints, the patient was willing for medical treatment. Respecting her wishes, the patient is continued on drug therapy for the past 1 year on which she is doing well.

Discussion

Right-sided valvular heart disease is often less regarded due to its uncommon isolated existence and prolonged latent asymptomatic period,⁷ tricuspid being even referred to as the ‘forgotten valve’.⁸ The combination of tricuspid valve with pulmonary valve involvement is not commonly described apart from carcinoid heart disease, which is characterised by deposition of carcinoid plaques over the endocardial surface of the cardiac valves. It is commonly associated with tricuspid valve insufficiency with pulmonary valve stenosis, and calcification is almost never seen.⁹

Other causes of tricuspid stenosis include drug intake, infective endocarditis or placement of pacemaker leads, however no such history could be elicited in our patient. In very rare cases, tricuspid stenosis may be the only manifestation of rheumatic heart disease¹⁰ or be associated with disseminated lupus erythematosus.¹¹

Contrarily, valvular pulmonary stenosis is often congenital in origin and may present with dyspnoea, fatigue or features of right-sided heart failure in older age groups.¹² Few studies however have reported the association of tricuspid valve calcification in patients with untreated long-standing pulmonary valve stenosis. Arnold et al described two cases of severe tricuspid valve calcification in association with congenital pulmonary valve stenosis in their fifth decade of life. However, despite having calcification, no gradient was found across the tricuspid valve.¹³ A similar case was described by Hollman et al with calcification of the tricuspid valve in postmortem specimen of a 46-year-old man with severe pulmonary valve stenosis.¹⁴ The severe calcification of tricuspid valve in both these cases was attributed to protracted increase in right ventricular pressures secondary to severe pulmonary stenosis, leading to degeneration and calcium deposition.

In our case, the tricuspid valve calcification can be a manifestation of long-standing increase in right ventricular pressure secondary to congenital pulmonary valve stenosis. However, previous case reports have not described associated haemodynamic repercussions of the affected tricuspid valve. Moreover, there was no history of any childhood heart disease in our patient. Tricuspid valve calcification also has not been described in other cases of severe pulmonary artery hypertension which may have similar haemodynamic stressors.

Thus, although exact aetiology could not be ascertained in our case, severe tricuspid valve involvement was likely a consequent to severe congenital pulmonary valve stenosis with increased right ventricular pressures leading to degeneration, calcification and subsequent stenosis. Unusual longevity and such presentation has not been described previously which adds up to the enigma of the case.

Patient’s perspective.

I feel satisfied that an appropriate diagnosis of my condition has been made and a treatment option by means of surgical correction is possible. Once adequate finances are arranged, I will get myself operated.

Learning points.

• Isolated right-sided valvular involvement, although rare, forms an important cause of right-sided heart failure.

• Tricuspid valve pathology needs to be better studied and understood. In the presence of multiple modes of imaging, it no longer should be considered a ‘forgotten’ valve.

• Natural history and uncommon presentation of congenital heart diseases should be considered while evaluating patients with valvular heart disease.