Eur J Pediatr (1992) 151 : 745-747

European Journal of

Pediatrics

9 Springer-Verlag 1992

Unsuspected rheumatic heart underlying group B streptococcal endocarditis at the age of 20 months L. Moral 1, J. Maid 3, E. M. Rubio I, C. Ruiz 3, and J. Casaidfiliga 2 1Servicio de Pediatria, 2Unidad de Cardiologfa Pedifitrica, Hospital Materno-Infantil Valle de Hebr6n, 3Servicio de Anatom/a Patol6gica, Ciudad Sanitaria Valle de Hebr6n, Paseo Valle de Hebr6n 119-129, E-08035 Barcelona, Spain Received October 17, 1991 / Accepted after revision February 26, 1992

Abstract. T h e case is r e p o r t e d o f a 2 0 - m o n t h - o l d girl adm i t t e d to o u r c e n t r e for g r o u p B s t r e p t o c o c c a l e n d o c a r ditis w h o d i e d of c o m p l e t e a t r i o v e n t r i c u l a r b l o c k a f t e r a w e e k of t r e a t m e n t . T h e r e was no h i s t o r y p o i n t i n g to the p r e s e n c e of a h e a r t d i s o r d e r . N e c r o p s y d i s c l o s e d the surprising e x i s t e n c e o f a vast r h e u m a t i c carditis involving e s s e n t i a l l y t h e a o r t i c a n d m i t r a l valves, with b a c t e r i a l superinfection of the former. The double rheumatic and infectious lesion in such a y o u n g p a t i e n t with infective p s e u d o - a n e u r y s m s o f t h e sinuses of V a l s a l v a m a k e s this an e x c e p e t i o n a l case.

Key words: R h e u m a t i c carditis - G r o u p B s t r e p t o c o c c a l e n d o c a r d i t i s - Sinus of V a l s a l v a a n e u r y s m - A t r i o v e n t r i cular block - Child

Introduction R h e u m a t i c f e v e r ( R F ) is n o w a d a y s a d i s e a s e with a declining i n c i d e n c e in o u r a r e a [4], a n d a l t h o u g h it is seld o m seen u n d e r the age of 3 years [12, 16], carditis reaches an i n c i d e n c e o f 90% in this age g r o u p [21], h i g h e r t h a n in o l d e r c h i l d r e n . A s c h o f f b o d y is its v i r t u a l l y p a t h o g n o m o n i c lesion [3, 7, 21]. W e r e p o r t an e x c e p t i o n a l case of r h e u m a t i c carditis ( R C ) d e t e c t e d on p o s t m o r t e m exa m i n a t i o n in a t o d d l e r with g r o u p B s t r e p t o c o c c a l ( G B S ) endocarditis (EC).

Case report This patient was a previously healthy 20-month-old girl. Within the previous 2 months, she had shown mild anorexia and weakness coinciding with attendance to a day nursery. Two days before hosCorrespondence to: J. Casaldgdiga

AV = atrio-ventricular; EC = endocarditis; GBS = group B streptococcus/al; RC = rheumatic carditis; RF = rheumatic fever

Abbreviations:

pitalization she developed progressive prostration and fever with absence of diuresis that determined admission to a local hospital, where diagnosis of aortic valve infective EC was made. After treatment of heart failure had been attained in a few hours on digoxin and furosemide, the child was transferred to our centre. Blood, urine and cerebrospinal fluid were cultured, but only the former was positive for GBS. Her physical examination included the following data: height 81cm (50th percentile) and weight 9.5 kg (10th percentile), temperature 37.3~ heart rate 130 beats/min, respiratory rate 40 breaths/min and blood pressure 120/70mmHg. She had a moderately ill appearance with intense mucocutaneous pallor. Peripheral pulses were prominent (water-hammer pulse). There were neither skin lesions nor arthiritis nor meningism. Her abdomen was not distended and the edges of her liver and spleen were palpable. A grade II/VI systolic ejection murmur, maximal at the upper right sternal border with a III/VI early blowing diastolic murmur best heard at the middle left sternal border were felt. Significant laboratory results included an haemoglobin concentration of 9 g/dl and a leucocyte count of 22.600/mm 3 with a shift to the left; a sedimentation rate of 52 mm/h and C-reative protein of 8.8 mg/l. A blood sample to determine ASO titres was insufficient in quantity for laboratory processing. There was mild radiological cardiac enlargement with normal ECG. An echocardiogram showed a dilated left ventricle, a mitral vegetation measuring 6 mm on the atrial side of the septal leaflet and severe destruction of the aortic valve. Doppler examination confirmed the severe aortic insufficiency and revealed a minimal mitral regurgitation. With the diagnosis of infective EC with mitro-aortic involvement, the child was given digoxin, furosemide and antibiotic treatment (penicillin G plus streptomycin). After admission her clinical condition remained unchanged until a sudden worsening occurred on day 7 due to complete atrioventricular (AV) block that led to her death despite resuscitation efforts.

Necropsy findings T h e m a i n findings w e r e l i m i t e d to the h e a r t , the w e i g h t o f w h i c h was 85 g, showing a situs solitus w i t h o u t a n y e v i d e n c e o f c o n g e n i t a l m a l f o r m a t i o n s . A t t e n t i o n was foc u s e d on two infective p s e u d o - a n e u r y s m s in b o t h coron a r y sinuses o f V a l s a l v a (Fig. 1). T h e t h r e e a o r t i c valve cusps w e r e i r r e g u l a r l y t h i c k e n e d d u e to f i b r o b l a s t i c p r o l i f e r a t i o n with n e c r o t i c m a t e r i a l a n d n u m e r o u s g r a m p o s i t i v e cocci on their surface.

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Fig. 1. Longitudinal section through the aortic wall (AO), sinus of Valsalva (ST/), aortic valve leaflet (AVL), and left ventricle (V), showing the aortic valvulitis and one of the infectious pseudo-aneurysms protruding into the aortic root (thick arrows). Note the communication between the pseudo-aneurysm and the aortic lumen across a mediointimal rupture (thin arrow) (Gomori trichromic • 4.5)

On the atrial side of the septal mitral leaflet there was a verrucous nodule of 6 mm in diameter, that microscopically exhibited the rheumatic characteristics [19] (Fig. 2). Sections throughout the heart revealed a patchy fibrinoid degeneration of collagen fibres in the endocardium, pericardium and the adventitia of the small intramyocardic vessels, accompanied by a mild infiltrate of lymphocytes, plasma cells, histiocytes and Anitschkow cells. Despite a large number of microscopic sections, we found just one Aschoff body (Fig. 3) according to the criteria proposed by Saphir [17]. Both papillary muscles of the mitral valve apparatus showed extensive acute myocardial infarcts. On the other hand, the A V node study revealed a granulomatous lesion involving the wall of the artery supplying this structure.

Discussion RF is rare under the age of 5 years, and particularly uncommon below 2 years. In the pre-penicillin era, McIntosh and Wood [12] compiled 2,884 cases of RF, 15 (0.5%) of them being children under 2 years of age. In the early-penicillin era, Rosenthal and coworkers [16] found, among 1,926 initial attacks of RF in children under

Fig. 2. Rheumatic verrucous nodule from the mitral valve showing neovascularization in a young connective tissue with round inflammatory cells and fibrin with Aschoff cells on the surface (arrow) (HE • 100) 17 years, just 3 (0.1%) younger than 2. In the 1970s, only 1 case aged less than 5 years (1-year-old) from a total of 31 was diagnosed in the whole of Sweden [18], and nobody was in that age range from a total of 115 cases during the past two decades in a hospital at New York [9]. Again, incidence and severity of RC under 3 years of age in RF patients seem to be greater than in the elderly, reaching 90% of prevalence [12, 16, 21]. The presence of an Aschoff body makes the diagnosis of RF unquestionable, since this lesion, although uncommon [15], is pathognomonic of this entity [3, 7, 21]. In our patient it escaped clinical notice because of the unusual establishment at a precocious age and the coincidence of a much more evident bacterial EC. With hindsight, the only fact suggesting RC would have been the concomitant involvement of both aortic and mitral valves, a reliable clue of rheumatic valve disease, as usually reported [7, 14]. Maintaining the criteria of Jones for the diagnosis of RF [2], the presence of one major (carditis) and two minor manifestations (fever and elevated acute phase reactants) were observed, although these findings are common to bacterial EC. There was no evidence of a previous pharyngitis, as occurs in at least one third of cases [3, 21]. Infection with group A Streptococcus could not be demonstrated because of the above problem. There is no identifiable source for the bacteraemia that originated the EC, as usually happens in paediatric patients [11]. Worthy of note is the isolation of GBS as the aetiological agent of the EC, a rare pathogen in this

747 OUS i n v o l v e m e n t of b o t h a o r t i c a n d m i t r a l valves t a k e s place.

Acknowledgement. We thank Dr. Luis Mir6 for the review of English translation.

References

Fig. 3. A Wide areas of fibrinoid necrosis of the endocardium (~r) located just under the mitral valve, where there was the only Aschoff body we could find (arrow) (HE x 25). B A higher magnification of this Aschoff body. It consists of Aschoff cells (arrows), Anitschkow cells and mononuclear cells. Fibrinoid necrosis is present adjacent to the nodule (HE • 250)

k i n d o f i n f e c t i o n in c h i l d r e n [1, 22]. Its invasive p o w e r has also b e e n r e p o r t e d r e g a r d i n g v a l v u l a r d e s t r u c t i o n , myocardial involvement, mycotic aneurysm development a n d m o r t a l i t y r a t e [1, 8, 13]. A r e m a r k a b l e finding was t h e f o r m a t i o n of an infective sinus of V a l s a l v a p s e u d o a n e u r y s m , an i n f r e q u e n t c o m p l i c a t i o n of b a c t e r i a l E C , p a r t i c u l a r l y in c h i d r e n [1, 10, 20]. N o n s u r g i c a l a c q u i r e d c o m p l e t e A V b l o c k is i n f r e q u e n t in c h i l d r e n , a n d b o t h R F a n d E C can c a u s e it. A special sensitivity to digitalis has b e e n r e p o r t e d in p a t i e n t s with r h e u m a t i c m y o c a r d i t i s [3], a n d t h e r e is s o m e p r o b a b i l i t y for this d r u g to b e r e l a t e d to t h e A V b l o c k t h a t l e d to o u r p a t i e n t ' s d e a t h . I n this r e s p e c t , an i n d e t e r m i n a t e r o l e c o u l d h a v e b e e n p l a y e d b y t h e c h r o n i c l e s i o n f o u n d on the nodal artery. T h e o c c u r r e n c e o f such a p e c u l i a r case is striking in the c u r r e n t e r a of s h a r p d e c l i n e in t h e i n c i d e n c e o f R F in o u r a r e a [4], a l t h o u g h a n e w rise has b e e n r e p o r t e d in s e v e r a l p a r t s o f N o r t h A m e r i c a [5]. T h e r e is at least o n e r e p o r t o f such a r e s u r g e n c e in E u r o p e [6]. W e h a v e p r o b a b l y w i t n e s s e d an a t y p i c a l case of t h e d i s e a s e , as m u c h for t h e y o u n g age o f its e s t a b l i s h m e n t as f o r its r e p o r t e d l o w i n c i d e n c e a n d t h e p r e c o c i o u s s u p e r i n f e c t i o n b y an u n u s u a l agent. P e r h a p s t h e exist e n c e of R C s h o u l d b e s u s p e c t e d in t h e face o f e v e r y E C d e v e l o p e d in t h e a b s e n c e o f c o n g e n i t a l h e a r t d i s e a s e , at w h a t e v e r age it a p p e a r s , e s p e c i a l l y w h e r e s i m u l t a n e -

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Unsuspected rheumatic heart underlying group B streptococcal endocarditis at the age of 20 months.

The case is reported of a 20-month-old girl admitted to our centre for group B streptococcal endocarditis who died of complete atrioventricular block ...
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