Rheumatic heart disease (RHD) is the most common cause of acquired heart disease in children and young adults worldwide and particularly developing countries continuing to experience a high incidence of this disease. The unexpected increase in the incidence of the disease in certain areas may explain the clinical and epidemiological characteristics of this disease. The key manifestation of RHD is the cardiac valvular abnormalities characterized principally by deforming the layered and avascular leaflet architecture due to inflammation and subsequent diffuse fibrosis. Mitral valve is mostly involved and pulmonary valve is rarely affected. Background of these case reports highlighted the increased incidence of rheumatic pulmonary valve disease in Thoothukudi region of India in Tamil Nadu state.
Rheumatic fever is the commonest form of heart disease in many developing countries of tropics and subtropics including Southern states of India. There are relatively few countries in the world without any data on rheumatic fever [
The literature has been reviewed repeatedly and recent reviews [
Rheumatic fever often produces a pancarditis, characterized by endocarditis, myocarditis and pericarditis. Endocarditis is manifested as valvular insufficiency and severe scarring of the valve develops during a period of months to years after an episode of acute rheumatic fever, and recurrent episodes may cause progressive damage to the valves. The mitral valve is affected most commonly and severely in 65% - 70 % of patients and the aortic valve is affected in 25%. The tricuspid valve is involved in only 10% of patients, almost always in association with mitral and aortic lesions. RHD is responsible to 99% of mitral valve stenosis in adults and the pulmonary valve is rarely affected.
An isolated rheumatic involvement of pulmonary valve is uncommon in literature and so it had been reported.
A 9 year old asymptomatic male child, having precordial murmur was referred for echocardiographic evaluation The child had 2 to 3 episodes of febrile illness with joint pains at the age of 3 - 4 years and not taken penicillin prophylaxis thereafter.. His pulse rate was 80 bpm. Blood chemistry revealed a positive ASO titer suggesting an antecedent streptococcal infection. ECG revealed the persistent juvenile pattern of T inversion in V1-V3 and X-ray chest revealed mild prominence of main pulmonary artery. Physical examination revealed normal general appearance with a grade 3/6 systolic murmur over the precordium, most prominent in the left second intercostal space and a phasic ejection click which was loudest during expiration and diminished in intensity during inspiration. The second heart sound was soft and single. These features were consistent with valvular pulmonary stenosis. Echocardiography revealed a thickened pulmonary valve as shown in
A 3 year old female child having grade 2/6 systolic murmur over the precordium was referred for screening echocardiopgraphy with a positive serum ASO titer. The child had a history of febrile illness during the neonatal period and the nature of illness was unknown. Pulse rate was 90 bpm. General appearance was normal and cardiac auscultation revealed 2/6 systolic murmur in the left second intercostal space. ECG and X-ray chest were normal. Transthoracic echocardiographic imaging revealed bright echoes on the tip of pulmonary valve with mild thickening suggest rheumatic inflammation as shown in
Diseases of the pulmonary valve are most often congenital, and only rarely due to acquired disorders such as carcinoid, rheumatic and infective endocarditis [
Rheumatic disease causing pulmonary valve stenosis is quite uncommon and, when it occurs, it is invariably associated with rheumatic disease of other cardiac valves. Vela and colleagues [
Isolated rheumatic involvement of pulmonary valve is observed in children < 15 years of age at the tropical zone of Thoothukudi in India. Majority are presented with mild to moderate pulmonic stenosis without the involvement of other cardiac valves. Earliest rheumatic involvement of the pulmonic valve is noticed frequently among school referral cases in this region.
Rheumatic fever is a non-suppurative sequelae to the rheumatogenic strains of Lancifield group A β hemolytic streptococcal infection of tonsillopharynx after a latent period of approximately 3 weeks and causing exudative and proliferative inflammatory reaction which in turn damage to collagen fibrils and ground substance of connective tissue. These rheumatogenic strains are often encapsulated mucoid strains, rich in M proteins and resistant to phagocytosis. These strains are strongly immunogenic and immunologic cross-reactions between the streptococcal carbohydrate and valvular glycoprotein, resulting damage to heart valves. Group A streptococci (GAS) elaborate the cytolytic exotoxins, streptolysins S and O that act as antigens and the affected individuals produce specific antibodies against these antigens. Of these two toxins, streptolysin O induces persistently high antibody titers that provide a useful marker of GAS infection and its nonsuppurative complications. The antibodies to the extracellular streptococcal antigens rise during the first month after infection and then plateau for 3 - 6 months before returning to normal levels after 6 - 12 months. When the ASO titer peaks (2 - 3 weeks after the onset of rheumatic fever), the sensitivity of the test is 80% - 85%. Several serological tests are now available to determine the occurrence of an antecedent streptococcal infection. Most of these tests assay for neutralizing antibodies to streptococcal extracellular enzymes. The first such test, described by Todd in 1932 [
Serum ASO titer (a non-type-specific antibody test) was positive in these children, suggesting the laboratory evidence of antecedent Group A streptococcal infection.
In individuals with rheumatic heart disease (RHD), echocardiography is useful to identify and quantify the valvular lesions. Studies in Cambodia and Mozambique have demonstrated a 10-fold increase in the prevalence of RHD when echocardiography is used for clinical screening compared with strictly clinical findings [
Most children with pulmonary stenosis do not require evaluation beyond echocardiography. Two dimensional and Doppler imaging is the sine qua non of its diagnosis and a thickened pulmonary valve with restricted systolic motion (doming) in the parasternal short axis view is the characteristic feature [
Color Doppler imaging shows smooth, laminar subpulmonary flow (blue) and some flow acceleration (red) immediately beneath the pulmonary valve with turbulent (mosaic) flow beginning immediately distal to the pulmonary valve leaflets as shown in
Valvular thickening has been demonstrated to be a feature of RHD on echocardiography and is often most marked at leaflet tips [
Therapy is directed towards preventing recurrent rheumatic heart disease in children and monitoring for the complications and sequelae of chronic rheumatic heart disease in adults. The importance of preventing recurrences of rheumatic fever is evident. The incidence of residual rheumatic heart disease at ten years is 34% in patients without recurrence and 60% in patients with recurrent rheumatic fever. Disappearance of the murmur, when it occurs, happens within 5 years in 50% of patients. Thus, significant number of patients experience resolution of valve abnormalities even 5 - 10 years after the episode of rheumatic fever. Oral penicillin V remains the drug of choice for treatment of streptococcal pharyngitis. Oral penicillin 250 mg for children and 500 mg for adults, given 3 times daily for 10 days is the primary recommended regimen. For recurrent pharyngitis, a second 10-day course of the same antibiotic may be repeated. GABHS (Group A beta hemolytic streptococcus) carriage is difficult to eradicate with conventional penicillin therapy. Thus, oral clindamycin (20 mg/kg/day) in 3 divided doses for 10 days is recommended. Although oral penicillin prophylaxis is also effective to prevent recurrent
episodes, data from the World Health Organization (WHO) indicate that the recurrence rate of GABHS pharyngitis is lower when penicillin is administered parentally.
In case-1, an injection of 1.2 million units of benzathine penicillin G intramuscularly every 3 weeks for this 9-year old boy and in case-2, 0.6 million units for this 3-year old female child are advised as a preventive and prophylactic therapy for a duration well into adulthood and preferably for life. Tompkin et al. [
In acute rheumatic heart disease, heart catheterization is not indicated. With chronic disease, heart catheterization has been performed to evaluate the valve disease and to balloon the stenotic valve. More recently, the interventional procedures are increasingly performed under general anaesthesia and the gradients are usually lower than those with conscious sedation. Therefore, the Doppler-echocardiography gradients should be used in making the decision regarding balloon pulmonary valvuloplasty and balloon dilation should be performed only in patients with peak-to-peak gradient of more than 50 mmHg. Patients with echocardiographic evidence of clinically significant pulmonary stenosis (50 - 60 mmHg) should undergo diagnostic and therapeutic cardiac catheterization with preparation for balloon dilation of the pulmonary valve [
The positive ASO titers of these cases indicate the recent streptococcal infection suggesting the recurrent episodes, both preventive and prophylactic therapies are indicated A peak gradient of 48 mmHg (PS velocity - 3.45 m/s) across the pulmonary valve suggesting moderate to severe stenosis and a pulmonary diastolic pressure of 9 mmHg (PR velocity - 1.51 m/s) on Doppler-echocardiography which is similar to catheter-based pressure in the pulmonary veins (PCWP = LA pressure) suggest an isolated pulmonary valve disease of rheumatic etiology as shown in
Handheld echocardiography has been investigated as a screening tool and found to be 90% sensitive and 92% specific for identifying patients with rheumatic heart disease in Uganda children [
Rheumatic involvement of pulmonary valve is frequently observed in the coastal district of Thoothukudi in India and an isolated rheumatic pulmonary valve disease in a 9 year old male and in a 3 year old female child is detected by Transthoracic echocardiographic imaging. Both echocardiography and ASO screening test, which are adapted to detect rheumatic cases and early administration of penicillin prophylaxis is practiced in positive cases as a preventive measure and penicillin therapy is given to treat any episodes of streptococcal pharyngitis which is endemic in this tropical region. From these case reports, it is known that a possibility of rheumatic pulmonary valve disease is more common similar to congenital etiology and more frequent like rheumatic mitral, aortic and tricuspid valve diseases in this region.
Ramachandran Muthiah, (2016) Isolated Rheumatic Pulmonary Valve Disease—Case Reports. Case Reports in Clinical Medicine,05,207-215. doi: 10.4236/crcm.2016.56039