|CURRICULUM IN CARDIOLOGY - CASE DISCUSSION
|Year : 2018 | Volume
| Issue : 1 | Page : 41-48
A case of rheumatic heart disease with history of complicated pregnancy
Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||4-May-2018|
Dr. Nirmal Ghati
6/2/4, Ramkali Mukherjee Lane, Kolkata - 700 050, West Bengal
Source of Support: None, Conflict of Interest: None
A 24-year-old female with rheumatic heart disease presents with a 6 years history of symptoms, with an eventful pregnancy, medical management, and subsequent surgery. The history and management is discussed.
Keywords: Bedside case, case discussion, rheumatic heart disease
|How to cite this article:|
Ghati N. A case of rheumatic heart disease with history of complicated pregnancy. J Pract Cardiovasc Sci 2018;4:41-8
| Presentation|| |
A 24-year-old lady, resident of Bihar, India, presented with shortness of breath for 6 years and intermittent palpitation for 2 years. She was apparently well 6 years back when she developed dyspnea on exertion in her 6th month of pregnancy. The dyspnea progressed to New York Heart Association (NYHA) IV over 1 month and she also started having orthopnea and anasarca at the end of 7th month of pregnancy. She was admitted and evaluated in a tertiary care hospital where she was diagnosed to have a valvular heart disease. She had to be induced for vaginal delivery at the 7½ months of pregnancy as she could not be managed medically. She delivered a healthy preterm baby and following the delivery she improved with conservative management. She was advised for a valve surgery and 3 weekly benzathine penicillin injection, but she did not undergo surgery due to poor financial status. After discharge, she continued to have the NYHA II symptoms. Three years later, she also stopped the penicillin injection without consulting any physician.
Two years back she started having intermittent palpitation and sense of irregular heartbeat which has a gradual onset and offset. She started taking medication for the palpitation which decreased her symptoms partially, but for again for the last 6 months, she is having excessive palpitation and NYHA III dyspnea in spite of medicines. There is a history (when she was 10 years old) of an episode of fever and migratory arthritis involving the knees, ankles, and elbows for 2 weeks. She carries some echocardiography reports and hospital papers of that illness which suggest that she was seen in a tertiary care hospital and was treated with steroids. She was also advised monthly injections which she took for 4 months and stopped.
On examination, the patient was tachypneic; blood pressure was 80/40 mmHg and heart rate was 110 bpm and irregularly irregular. Jugulo venous pressure (JVP) was raised, and “a” waves were absent. There was no pallor, icterus, or pedal edema. Lung fields were clear, and there was no hepatomegaly.
On cardiac examination, there was Grade II parasternal heave. The cardiac impulse was displaced to the sixth intercostal space and 2 cm outside the midclavicular line, and it was hyperdynamic in nature. S1 was loud and variable in intensity and S2 was narrow split with loud P2.
There was a Grade III pansystolic murmur at the apex going to the axilla. There was also a long mid-diastolic rumble with no presystolic accentuation. It was associated with a diastolic thrill. There was also an early diastolic high-pitched murmur at the 2–3rd intercostal left spaces which was occupying more than half of diastole and starting with A2. There was also a Grade II, pansystolic murmur increasing with inspiration audible at the lower left sternal border.
Her electrocardiography revealed atrial fibrillation with fast ventricular rate. CXR showed cardiomegaly with biatrial enlargement [Figure 1] and [Figure 2].
|Figure 1: Electrocardiography showing atrial fibrillation with fast ventricular rate|
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|Figure 2: Chest X-ray showing cardiomegaly, left atrial enlargement, pulmonary venous hypertension|
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Echocardiography revealed rheumatic heart disease (RHD) [Figure 3], moderate-to-severe mitral regurgitation (MR), moderate MS, moderate-to-severe aortic regurgitation (AR), hypertensive tricuspid regurgitation, severe pulmonary arterial hypertension, and mild left ventricular (LV) dysfunction (LV ejection fraction = 50%–55%). She was initially managed with intravenous diuretics, beta-blocker, oral digoxin, and oral anticoagulant. She underwent aortic and mitral valve replacement successfully and her postoperative days were uneventful.
|Figure 3: Echo (apical 4 chamber view) showing posteriorly directed eccentric jet moderate-to-severe mitral regurgitation and dilated left atrium|
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| Relevant Questions|| |
(Q1) What was the episode at the age of 10 years? Discuss the first possibility and the differential diagnosis
At the age of 10 years, she had a fever with migratory arthritis of the large joints. The first diagnosis we would think of is the acute rheumatic fever (ARF). Arthritis is one of the common major manifestations of ARF, and it is characterized by sequential involvement of large joints (mainly knees and ankles). Although classically described as “migratory polyarthritis,” simultaneous involvement of multiple large joints is not infrequent. It may present as monoarthritis, especially if anti-inflammatory therapy is started at an early stage. Arthritis frequently co-exists with carditis, and its severity is usually inversely related to carditis severity. In one study, severe carditis was present in 10% cases with arthritis. In comparison, severe cardiac involvement was present in 33% patients with arthralgia and 50% patients with no joint symptoms. Alternate possibilities to be ruled out in children are systemic-onset juvenile rheumatoid arthritis, SLE, Henoch–Schonlein purpura, septic arthritis, etc., each having distinguishing features and investigations for diagnosis [Table 1].
|Table 1: Differential diagnosis of arthritis of acute rheumatic fever and their distinguishing features|
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(Q2) What are the predominant valvular defects of acute rheumatic fever? Discuss their natural history
Although classically described as pancarditis, endocarditis in the form of valvular involvement is the most important manifestation of acute rheumatic carditis. MR is seen in 70%–75% of the patients. Most commonly MR is of mild-to-moderate severity, but severe MR with congestive heart failure may occur occasionally., The factors leading to MR in ARF are inflammatory valvulitis, valve prolapse, annular dilatation, ventricular enlargement, and ruptured chordae. Mitral plus AR seen in 20%–25% patients and isolated AR is found in 5%–8% of patients. AR in ARF is most commonly mild to moderate in severity. Valve stenosis does not occur in ARF setting. Clinical tricuspid valve involvement is rare during the first attack of rheumatic fever, but pathologic studies have reported tricuspid valve involvement in 30%–50% of patients. Pulmonary valve defect is rare both clinically and pathologically.
Among the clinical manifestations of ARF, carditis is the most important prognostic marker. Hence, an in-depth knowledge of natural history of rheumatic carditis is invaluable in designing proper follow-up strategy, timely intervention, and implementation of prophylaxis. As natural history study in the present era is not possible due to ethical issue, the wealth of our knowledge comes from older studies. An observational study in prepenicillin prophylaxis era is invaluable in this regard. In this study, a total of 1000 ARF patients (mean age ~8 years) were followed up for 20 years. At the start of the study, 653 (65.3%) patients had residual valve defect and 347 (34.7%) did not have any clinical RHD. Among those who had valve defect at the start of the study, the physical signs of RHD disappeared in 11% cases after 10 years and in 16% cases after 20 years of follow-up. On the other hand, new features of RHD appeared in 24% cases after 10 years and in 44% cases after 20 years of follow-up; those patients who were clinically recovered the rheumatic fever attack. Pure mitral stenosis (MS) was the predominant valvular lesion in those patients. The evolution of MR of ARF was interesting. In 33% cases the murmur disappeared, in 40% cases it persisted, and in 19% cases, additional diastolic murmur of MS appeared. In 8% cases, it converted to pure MS. MS develops with a latent period of 5–20 years after first rheumatic fever attack. In the study by Bland and Duckett Jones, pure MS developed in 11.7% cases of ARF after 20 years of follow-up and 35.8% of them were clinically asymptomatic at the start of the study. Less than 10% patients with MR and severe carditis during initial rheumatic fever develops MS on follow-up. History of ARF can be found in lesser percentage of patients (51%) with isolated MS than in those who have MR. In addition to MR, AR appears in 20%–25% cases of ARF, and at the end of the third decade, nearly 58% patients develops AR. Isolated AR is an unusual finding (~7% cases). In the Bland and Duckett Jones study, aortic stenosis developed with AR in approximately 7% cases at the end of the third decade of age. Pure aortic stenosis is infrequent under 30 years of age and when it occurs bicuspid aortic valve should be ruled out.
(Q3) What is “juvenile mitral stenosis?” How is it different from adult rheumatic heart disease?
The natural course of MS is different from MR. MS during acute rheumatic carditis is an unusual finding. Observational studies of the natural history of pure or predominant MS described average 20 years of latent period between first rheumatic carditis attack and clinically severe MS.,, However, the evolution of MS is different in some part of the world like India where symptomatic critical MS can appear at much younger age (often < 12 years). Roy et al. first identified and studied these patients and coined the term “juvenile MS.” In the same series, 40% of the patients had pure MS and additional 33% patients had MS with mild MR. History of one attack of ARF was present in 66% patients and more than one attack was present in 28% patients. Apart from the younger age of onset, these patients behave differently than usual cases of MS. Boys are more commonly affected. Mitral valve in these patients is usually thickened and associated with severe subvalvular disease though calcification is rare. Due to less duration of illness, atrial fibrillation is an uncommon (~6%) complication. Severe pulmonary artery hypertension is found in two-third of these patients. Histopathologically, there is prominent medial muscular hypertrophy and intimal proliferation of pulmonary arteries accompanying the terminal and respiratory bronchioles. These patients frequently (~45% cases) present with congestive heart failure. Although success rate of balloon mitral valvotomy is similar to adult cases, relapse rate may be higher, especially in those who have active disease at the time of the procedure.
(Q4) What is the current reported incidence and prevalence of acute rheumatic fever and rheumatic heart disease in India?
In 1870, WJ Moore first reported numerous cases of RHD from Rajasthan, India, but the first clinical evidences of rheumatic fever and RHD came from studies done by Wig in 1935 and Kutumbiah in 1940., Since then, several groups of investigators conducted studies to establish the burden of RHD in India. These studies can be divided into three groups – hospital data, population-based surveys, and school surveys. According to several hospital-based surveys, 20%–50% of hospital admissions are due to RHD patients. Although relatively easy to conduct, these hospital-based surveys give estimate of only the highly affected patients in the community. Several population-based surveys have also been conducted in several parts of the country, and they reported a prevalence of 0.2–2.2/1000 populations for RHD and 0.0007–0.2/1000 populations for rheumatic fever. Most of the information on the trend of RHD in India is found from school-based surveys. These studies were conducted in various part of the country with different methodology leading to significant difficulty in interpretation of the epidemiological trend. The reported prevalence varies from as low as 0.67/1000 school-aged children in Rajasthan to 2.1/1000 children in Punjab., The ICMR has conducted three school-based surveys. The first study (1972–1975) included 133,000 school children from Agra, Alleppey, Bombay (Mumbai), Delhi, and Hyderabad and reported overall prevalence of 5.3/1000 children (0.8–11/1000 children). The second study (1984–1987) included schools in Delhi, Varanasi, and Vellore and they reported an overall prevalence of 2.9/1000 children (1.0–5.6/1000 children). The third and the largest study included 176,904 school children from Shimla, Jammu, Chandigarh, Jodhpur, Indore, Kochi, Wayanad, Mumbai, Vellore, and Dibrugarh and the overall prevalence was 0.9/1000 children (0.13–1.5/1000 children)., The ICMR studies showed a decline in the prevalence of RHD in India. It may not be fully true because most of the severely affected underdeveloped parts of the country were not included in those studies.
(Q5) What is the role of echocardiography in the diagnosis of acute rheumatic fever? What is definition and prognosis of subclinical carditis?
Classically, carditis in ARF has been described as the presence of MR and/or AR murmur and presence of cardiomegaly in chest X-ray. Since advancement in echocardiography techniques, multiple studies have described a significant fraction of ARF patients where auscultatory findings of valvar dysfunction either were not present or were not recognized by the diagnosing clinician, but echocardiography/Doppler studies revealed mitral or aortic valvulitis., This entity is called subclinical carditis. As the prevalence of echocardiographically detected physiological MR and AR in normal population is up to 45% and 33%, respectively, the World Heart Federation has proposed a definitive criteria for the diagnosis of pathological AR and MR [Table 2].,, Till now, more than 25 studies have evaluated the prevalence of subclinical carditis in various population. According to one meta-analysis of 23 studies, the reported prevalence of subclinical carditis ranged from 0% to 53%. The pooled prevalence of subclinical carditis was 16.8% (95% confidence interval [CI] 11.9–21.6). This prevalence rate increased slightly to 18.1% (95% CI 11.1%–25.2%) when analysis was done on ten studies that applied full World Health Federation criteria for subclinical carditis diagnosis. On analyzing 11 studies which followed up patients with subclinical carditis, the pooled prevalence of persistence or deterioration of subclinical carditis was 44.7% (95% CI 19.3%–70.2%). One recent study has reported 28.2% prevalence rate of subclinical carditis among 103 patients who had carditis during ARF. After 1-year follow-up of 20 patients with subclinical carditis, the recovery and improvement rates were 15% and 30%, respectively. Due to the high prevalence rate and nonbenign nature, the American College of Cardiology/American Heart Association recently added subclinical carditis as a major entity in the revised Jones criteria for ARF. However, the studies which evaluated the prognosis of subclinical carditis followed up very less number of patients. The details of secondary prevention and degree of worsening have not been mentioned in most of the studies. Hence, larger follow-up studies are needed in the future to clarify the prognostic importance of subclinical carditis.
|Table 2: The World Health Federation criteria for the diagnosis of pathological mitral and aortic regurgitation|
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(Q6) What are the latest modifications in the diagnostic criteria of acute rheumatic fever?
Since the first criterion to diagnose ARF was proposed by Dr. T. Duckett Jones in 1944, it has been modified several times by the AHA and WHO. The latest modification by the AHA was published in 2015 [Table 3]. The revised model has separate criteria for high- and low-risk population. The high-risk population has been defined as ARF incidence ≥2/100,000 school-aged children or all-age RHD prevalence of ≥1/1000 population per year. One of the highlights of these revised criteria is the inclusion of subclinical carditis as a major criterion. Before that, only Australian and New Zealand guidelines used the subclinical carditis as a major criterion to diagnose rheumatic fever.,
|Table 3: The revised Jones criteria for diagnosis of acute rheumatic fever|
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(Q7) What is the role of steroid in the treatment of acute rheumatic carditis? Does it prevent permanent valve damage?
The main strategies of acute rheumatic carditis management are – bed rest, assessment of severity by clinical examination and echocardiography, management of heart failure, and other complications when present followed by secondary prophylaxis. The role of steroid (like other anti-inflammatory therapy) in ARF is controversial. The concept of cross-reactivity and aberrant immunological response to Group A streptococcal antigen in the pathogenesis of ARF makes the use of steroid a logical choice. Several reports published between 1950 and 1980 supported the use of the steroid as it decreases inflammation, resolves toxic state and murmur faster, prevents the development of new murmur, and helps resolution of pericardial effusion.,,,,,,,,,,, These encouraging reports led to eleven randomized trials which compared the effect of steroid versus placebo or salicylates in acute rheumatic carditis. None of the major trials showed any significant role of steroid in the treatment of acute rheumatic carditis and prevention of permanent valvular damage. Recently, one meta-analysis included eight randomized trials involving 996 patients with rheumatic fever. There was no significant difference in residual valvular damage at 1 year between corticosteroid and aspirin-treated group. However, we should not make a discouraging conclusion from these evidence because most of the trial was underpowered, old, used varying methodology, and included fewer patients with severe carditis, in which most of the benefit of steroid is expected. In many parts of the world, steroid (prednisolone 1–2 mg/kg/day) is still commonly used in severe carditis for faster recovery. It is usually continued for 4 weeks with a strategy to taper the dose gradually after resolution of the acute symptom (usually after 2 weeks).
(Q8) What is the role of secondary penicillin prophylaxis in prevention of rheumatic heart disease?
The first attack of rheumatic fever makes an individual susceptible to further recurrences. The recurrence rate is low in patients who escaped cardiac involvement during initial attacks. Recurrences are benign in these patients as there is a rare chance of cardiac damage with recurrent attacks. However, the prognosis is not so benign in patients with cardiac involvement during initial attacks with reported recurrence rate up to 40%–60%. Furthermore, each recurrence attacks are associated with additive cardiac damage leading to severe symptomatic RHD at an early age. Hence, prevention of recurrent Group A beta-hemolytic Streptococcus (GABHS) pharyngitis is the most effective way to prevent recurrent rheumatic fever attacks and RHD. Efforts to prevent recurrences of ARF started in 1936 with daily oral sulfonamide. Although several studies showed success with this prophylaxis, it lost popularity because of frequent hematological side effects and drug resistance. The next antibiotic which gained popularity and agent of choice for secondary prophylaxis till now is benzathine penicillin G (BPG). The first study which compared BPG with oral penicillin and sulfonamide was published in 1952 and with more comprehensive data in 1955., Although the recurrence rate of GABHS pharyngitis and RF was not statistically different between the groups, intramuscular BPG led to improvement in GABHS carriage rate and progression of detectable heart murmur. Another study in 1964 demonstrated RF recurrence rate of 0.4/100 patients years with intramuscular BPG injection, in comparison to 5.5/100 patients-years for oral penicillin and 2.8/100 patient-years for oral sulfonamide. GABHS pharyngitis rate was significantly low in patients with good adherence to prophylaxis. Regular BPG prophylaxis is associated with significantly less chance of progression to RHD in patients with mild carditis during the first attack of rheumatic fever. Furthermore, one study from India showed that 33% of the child with carditis during ARF had a resolution of cardiac murmur after 5 years of BPG prophylaxis. In 2002, a meta-analysis of 11 relevant trials published between 1959 and 1996 concluded that intramuscular BPG prophylaxis is associated with 71%–91% reduction in GABHS pharyngitis and 87%–96% reduction in rheumatic fever recurrence in comparison to oral penicillin prophylaxis. Although these reports are encouraging, most of the major trials were conducted in preechocardiography era and analyzed the efficacy of secondary prophylaxis using GABHS pharyngitis and RF recurrence rate as main surrogate markers. Randomized control trials with echocardiography to detect disease progression are needed in the future for better understanding of the efficacy of penicillin in secondary prophylaxis of RHD.
(Q9) Is 3-weekly penicillin prophylaxis better than 4-weekly prophylaxis? What is the current recommendation?
After the intramuscular injection of benzathine penicillin, serum levels of penicillin remained above the minimum inhibitory concentration for group A streptococci for 3–4 weeks. But according to some reports, the serum level of penicillin drops below effective level beyond 3 weeks of injection., There are few studies which showed the 3-weekly regimen or even 2-weekly regimen is better than 4-weekly regimen for the prevention of RF recurrences.,, In one study from Taiwan, the 3-weekly regimen resulted in greater resolution of MR (66% vs. 44%). However, the disadvantages of frequent dosing are frequent painful injection, increased cost, and decreased compliance. Hence, most of the current guidelines recommend empiric 4-weekly regimen with consideration for escalation to 3-weekly regimen in high endemic regions and with proven recurrence with 28-day regimen., Because of high disease burden in India, the Indian Academy of Pediatrics guideline has recommended a 3-weekly regimen for those taking 1.2 million unit dose (>27 kg) and a 2-weekly regimen for patients taking 0.6 million unit dose (<27 kg).
(Q10) What cardiac conditions can worsen in the 2nd trimester of pregnancy and warrant an induction?
Pregnancy leads to several adaptive changes in the maternal cardiovascular system. The cardiac output increases by 30%–50% during the first trimester and continues its increase until the second and third trimesters. Circulating blood volume reaches a maximum of 40% above baseline at 24 weeks' gestation. Moreover, cardiac output increases by another 15% in early labor, by 25% during stage 1, and by 50% during expulsive efforts of second stage of labor. The basal heart rate also rises by 10–30 beats/min late in pregnancy. All these changes can worsen preexisting cardiac diseases mainly starting at the second trimester of pregnancy. The cardiac conditions can be stratified according to the maternal and fetal risk using the modified World Health Organization approach [Table 4]., The task force on the management of cardiovascular disease in pregnancy from the European Society of Cardiology recommends intensive care for the WHO class III and Class IV conditions because of increased risk of premature induction of labor, morbidity, maternal, and fetal mortality.
|Table 4: The modified World Health Organization approach for stratification of cardiac condition which can complicate pregnancy|
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(Q11) What is the better mode for delivery in rheumatic heart disease? Lower segment cesarean section or vaginal delivery?
The preferred mode and timing of delivery depend on maternal and fetal hemodynamic status as well as obstetric indications. The decision should be taken by a multidisciplinary team that consists of the patient's obstetrician, obstetrical anesthesiologist, and a cardiologist. Vaginal delivery is the preferred mode of delivery in most women including patients with RHD. The advantages of vaginal delivery include less blood loss, an absence of abdominal surgery, more rapid recovery, and decreased thrombogenic risk. Moreover, the second stage of labor can be assisted with forceps or vacuum in women who cannot tolerate a prolonged second stage of labor or excessive maternal efforts with the Valsalva maneuver for cardiac reasons. Cesarean delivery eliminates the hemodynamic insults associated with labor, but it is associated with more significant blood loss and more abrupt hemodynamic changes, increased risk of venous thromboembolism, infection, and postpartum hemorrhage. The conditions where cesarean delivery is the preferred choice are pregnant patients with severe heart failure, aortic root >45 mm, acute aortic dissection, or patients on oral anticoagulants in preterm labor.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2], [Table 3], [Table 4]