Atypical presentation of rheumatic carditis in pregnancy

Abstracts

Descreve-se o caso de uma mulher de 31 anos de idade que, no decurso da segunda gestação, apresentou quadro atípico de doença reumática na 28ª semana. Aspectos relacionados à prevenção da moléstia e ao tratamento no ciclo gravídico-puerperal são discutidos.

Febre reumática; insuficiência da valva tricúspide; gravidez

Se describe el caso de una mujer de 31 años de edad que, en el transcurso de la segunda gestación, presentó cuadro atípico de enfermedad reumática en la 28ª semana. Se discutirán los aspectos relacionados a la prevención de la molestia y al tratamiento en el ciclo grávido-puerperal.

Fiebre reumática; insuficiencia de la válvula tricúspide; embarazo

CASE REPORT

Atypical presentation of rheumatic carditis in pregnancy

Lurildo Cleano Ribeiro Saraiva; Aluísio Macedo; Ana Elizabeth M. Batista

Serviço de Cardiologia da Universidade Federal de Pernambuco, Recife, PE - Brazil

Mailing address

SUMMARY

We report the case of a 31-year old woman presenting with atypical rheumatic disease in the 28th week of the second pregnancy. Questions related to prevention of the disease and its treatment during the pregnancy-puerperium cycle are discussed.

Key Words: Rheumatic fever; pregnancy; tricuspid valve insufficiency.

Introduction

The endemic persistence of rheumatic disease (RD) in Pernambuco has resulted in the emergence of unusual clinical forms in more developed regions, as observed for instance for rheumatic pneumonia¹.

With a prevalence of 0.65%, rheumatic disease is very rarely diagnosed in pregnant women, allowing the investigation of specific aspects relative to its prevention and treatment². The present work aims to report a case of possibly recurrent rheumatic disease during the pregnancy-puerperium cycle.

Case report

Thirty-one-year old woman from Recife, presenting with rheumatic heart disease, until then asymptomatic, with complaints of progressive dyspnea on exertion and tachycardia/palpitations beginning in the 28^th week of pregnancy. She reported having had a sore throat and hoarse voice around the 20^th week of pregnancy, which lasted for four days, despite the regular use of penicillin benzathine every three weeks. The patient had developed migratory arthritis of major joints at age 13, following an oropharyngeal streptococcal infection. When she was 19 years old, she had a recurrent arthritis on the 12^th day of puerperium, when a heart murmur was detected. During adolescence, the secondary prophylaxis of RD presented gaps.

Previous to the current pregnancy, she had had a clinical-echocardiographic diagnosis of mild double mitral lesion and aorta failure. Radiographic evaluation of the heart (fig. 1A) showed a normal heart area, with a cardiothoracic index of 0.44.

Examination of the patient showed, beside pregnancy, tachypnea and a mild lower limb edema. The following data were collected: AP = 130 x 70 mmHg; heart frequency of 120 beats/min; regular cardiac rhythm (B3) with a loud holosystolic murmur over the mitral area (MA) and an intense systolic murmur in the tricuspid area (TA); and moderate painful hepatomegaly. The echodopplercardiographic evaluation was compatible with severe mitral failure and mild tricuspid failure. The hemogram showed hypochromic anemia (hemoglobin = 11.1 g/dl), moderate leukocytosis (13,000/mm³) and accelerated erythrocyte sedimentation rate (37 mm/1^st hour). Normal levels of antistreptolysin O (AEO) were observed. The patient was treated with digital and diuretic agents, showeing improvement. After caesarean delivery and hospital discharge, the medication was suspended.

On the second puerperal month, the patient was received in the service in an urgency situation, presenting dyspnea, lower limb edema, prominent jugular stasis, precordial pressure compatible with hypertrophic growth in both ventricles, heart auscultation showing a gallop rhythm (B3), a severe holosystolic murmur in the MA and a musical systolic murmur in the TA. The liver was palpable at 8 cm from the right costal margin. The thorax radiography showed severe cardiomegaly (fig. 1B), and the electrocardiography revealed overload of the four heart chambers. The echodopplercardiography (fig. 2) showed thickened mitral, aortic and tricuspid valves, with a rheumatic aspect. Left ventricle (LV) ejection fraction was 64%. The LV diastolic and systolic diameters were 6.0 cm and 3.8 cm, respectively, and the estimated systolic pressure of the pulmonary artery was 54 mmHg.

All analyses of inflammatory activity had normal results, except for ultrasensitive C-reactive protein levels which were above normal (1.72 mg/dl, normal value < 0.50 mg/dl). AEO level was 59 UI/ml.

Following treatment with digoxin, furosemide and captopril, the physical capacity of the patient was normalized, and heart frequency was back to normal levels. The associated use of 30 mg prednisone resulted in recovery of heart failure (HF), and congestive symptoms disappeared after 20 days.

Discussion

The incidence of acute rheumatic disease during pregnancy is very low, and of difficult diagnosis. Inflammatory reactions, usually of a non-specific nature, are still less specific during pregnancy, when they are generally more severe². Hypervolemia and anemia, inherent to the condition, may simulate HF in a patient with established rheumatic valvular disease. In the present case, however, the patient had moderate valvular disease before pregnancy, but after delivery presented a severe mitral valvulopathy, aggravated by tricuspid failure which she did not have previously. Pregnancy does not result in anatomical lesions of human cardiac valves (fig. 2). In cases of stenosis, on the other hand, the dyspnea may become progressively more severe during pregnancy and improve after delivery. In the present case, HF appeared around the 7^th month of pregnancy, after a process of oropharyngeal inflammation, was followed by a new valvular lesion and resulted, in the puerperal period, in severe cardiomegaly (fig. 1B).

Two instructive aspects may be stressed in this case. The first one is related to recurrence of the RD in two different periods of the pregnancy-puerperium cycle. The second aspect is duration of prophylaxis for rheumatic disease, which recurred when the patient was 31 years old, age at which it is generally agreed that secondary prophylaxis for the disease should have ended.

The susceptibility of the pregnant patient to oropharyngeal streptococcal infection cannot be presently explained, since penicillin benzathine was used as recommended with intervals of 21 days³. Interestingly, this susceptibility was not followed by increased serum levels of antistreptolysin O, as expected in this type of situation due to the increasing severity of recurrent cases⁴.

Recent reports of RD during pregnancy are scarce, which is partly explained by a slight decrease in the incidence of the disease in our population. Series of isolated cases are described in the literature². In a review by Décourt (1986)², some aspects pertinent to the present case were emphasized, such as the usual recurrence of the disease, the difficulty experienced by patients in identifying their own HF symptoms, and the careful treatment with antiinflammatory drugs.

The use of prednisone to treat our patient may by discussed, since the evaluation of inflammatory activity had generally normal results, even when pregnancy may lead to modifications in the serum reactions during the acute phase². The many clinical signs suggesting severe cardiopathy, cardiomegaly (fig. 1B), the high C-reactive protein levels and the acute valvular involvement shown by echocardiography, particularly affecting the tricuspid valve (fig. 2), have however indicated its use. Prudent doses were used, considering that the patient was nursing and the baby could be affected. The results were positive, with restoration of good physical conditions to the patient in a short period. The patient remains asymptomatic.

Acknowledgment

We are indebted with Professor Edward Kaplan, from University of Minnesota Medical School, for valuable suggestions.

Potential Conflict of Interest

No potential conflict of interest relevant to this article was reported.

Sources of Funding

There were no external funding sources for this study.

Study Association

This study is not associated with any post-graduation program.

References

• 1. Saraiva LR. Rheumatic pneumonia (Letter to editor). Ann Rheum Dis. 2002; 61 (5): 477.
• 2. Décourt LV. Doença reumática ativa durante a gravidez. In: Lopes AC; Delascio D. (eds). Cardiopatia e gravidez. São Paulo: Sarvier Editora; 1986. p.113-21.
• 3. Décourt LV. Doença reumática. In. Décourt LV (ed). Medicina preventiva em cardiologia. São Paulo: Sarvier Editora; 1988. p. 101-17.
• 4. Chopra P, Gulwani H. Pathology and pathogenesis of rheumatic heart disease. Indian J Pathol Microbiol. 2007; 50 (4): 685-97.

Publication Dates

• Publication in this collection
  23 June 2009
• Date of issue
  Apr 2009

History

• Reviewed
  22 June 2008
• Received
  12 May 2008
• Accepted
  25 Aug 2008