134 Journal of Paediatrics and Child Health 44 (2008) 134–137© 2007 The Authors

Journal compilation © 2007 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

doi:10.1111/j.1440-1754.2007.01214.x

Key Points

1 Pulmonary oedema in acute rheumatic fever is likely due torupture of chordae tendinae of the mitral valve without leftatrial dilatation.

2 Such cases are often misdiagnosed as acute pneumonia.

3 Urgent cardiac surgery may be life-saving.

Correspondence: Dr Nigel Wilson, Paediatric Cardiac Services, StarshipChildren’s Hospital, Private Bag 92 024, Auckland, New Zealand. Fax:+64 9631 0785; email: nigelw@adhb.govt.nz

Accepted for publication 3 July 2007.

ORIGINAL ARTICLE

Fulminant mitral regurgitation due to ruptured chordae tendinae in acute rheumatic feverYvonne Anderson,1 Nigel Wilson,2 Ross Nicholson1 and Kirsten Finucane2

1Kidz First Children’s Hospital, Middlemore Hospital, South Auckland and 2Paediatric Cardiac Services, Starship Children’s Hospital, Auckland, New Zealand

Aims: Description of the presentation and management of cases of fulminant mitral regurgitation in acute rheumatic fever (ARF).Methods: Retrospective case series of 4 children, aged 6–10 years, presenting in acute pulmonary oedema because of rupture of elongationof the chordae tendinae of the mitral valve leading to flail leaflets and severe mitral regurgitation.Results: Urgent cardiac surgery with mitral valve repair was performed. Resolution of heart failure was achieved in each case. The difficultiesin diagnosis and management of this uncommon and often unrecognised presentation of ARF are discussed.Conclusions: Cardiac surgery can be life saving for fulminant mitral regurgitation due to rupture of chordae tendinae of the mitral valve in ARF

Key words: acute rheumatic fever; mitral regurgitation; ruptured chordae tendinae.

three of the four patients prior to surgery. Three of the fourpatents were managed with mechanical ventilation and thefourth was in respiratory failure despite high dose diuretics andoxygen prior to surgery (Table 2). The indication for surgicalintervention was deterioration despite maximal medical treat-ment in the intensive care setting.

The surgical and histological findings, type of operation andoutcome are outlined in Table 2. The mitral valve chordae ten-dinae were significantly elongated or ruptured in all cases withsubsequent flail segments of the mitral valve leaflets which hadresulted in torrential MR. In all four cases, mitral valve repairwas undertaken but two patients have subsequently requiredmitral valve replacement for recurrent progressive MR.

Discussion

The classical signs of rheumatic ruptured chordae tendinae arepulmonary oedema in the presence of severe MR because ofacute mitral valve prolapse. The left atrium is relatively non-compliant and this leads to a rapid rise in LA pressure, pulmo-nary venous pressure and overt pulmonary oedema. Thejugular venous pressure may also be raised as the V waveis transmitted through the right atrium.6 Breathlessness ismarked, and the acute pulmonary oedema is evident by bloodstained sputum, seen in two of the cases. Initially, there is noor minimal LA enlargement on ECG, chest X-ray and echocar-diography. In the presence of severe MR, this finding aloneshould alert one to the possibility that this is an acute problem,likely because of mechanical disruption of the mitral chordaetendinae. The left ventricle will dilate more quickly than the leftatrium, but this is much more modest than in classical severeMR in ARF that evolved over weeks and months.

Pulmonary oedema may be patchy or one-sided, and this hasled some authors calling the condition, wrongfully in our view,‘rheumatic pneumonia.’7 Clinicians and paediatricians assessing

Physicians)? 2007443134137Original ArticlesFulminant acute rheumatic feverY Anderson et al.

Rupture of chordae tendinae secondary to acute rheumaticfever (ARF) as part of the acute presentation phase was firstreported in 1968,1 and in a series of four patients in 1986.2 Sincethen single cases only have been reported.3–5 We report fourchildren with rheumatic fever presenting to one hospital inNew Zealand over a five month period with chordal rupture orelongation leading to severe mitral regurgitation (MR) andpulmonary oedema.

Patients

Each of the four patients initially presented to the Accident andEmergency Department of the Kidz First Children’s Hospitalwith the symptoms and signs of respiratory distress as outlinedin Table 1. Patient two had a history of respiratory distress, buton arrival at the Emergency Department, had depressed levelof consciousness and decreased respiratory effort.

All had relatively normal left atrial (LA) and left ventricular(LV) size on electrocardiography (ECG). Rupture or lengtheningof the chordae tendinae was recognised by echocardiography in

Y Anderson et al. Fulminant acute rheumatic fever

Journal of Paediatrics and Child Health 44 (2008) 134–137 135© 2007 The AuthorsJournal compilation © 2007 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

children may be reluctant to make the diagnosis of pulmonaryoedema in children but understanding the pathophysiology ofthis entity can enable the correct diagnosis in regions wherethere is rheumatic fever.

This series of children is older than that presented by deMoor,2 and surgical intervention was performed earlier. This ispartly because of experience of valve repair at our institution.The cardiac diagnoses were all made by echocardiography andno patient underwent cardiac catheterisation. However, in eachcase, there were still diagnostic challenges, with misinterpreta-tion of clinical and radiographic pulmonary oedema as pneu-monia by the emergency clinicians. Since De Moor’s report in1986,2 there have been advances in mitral valve repair and themajority of patients with rheumatic mitral prolapse are amena-ble to valve repair.8–10 Surgical techniques include shortening ofstretched tendinae or insertion of gortex chords to correct pro-lapse. The degree of annular dilatation is less in acute cases butannuloplasty may still be used. There will be ongoing inflam-

mation after surgery in these fulminant cases of ARF and alimited annuloplasty helps to prevent dilatation of the posteriorannulus and preserve the oval shape of the mitral annulus.Conservation of the mitral valve is now possible in most chil-dren requiring surgery for ARF but the difficulty of surgicalintervention because of the friability of the inflamed tissuemakes this a challenge. Because of the ongoing inflammationof rheumatic carditis which can last for 3–6 months,11 it is notsurprising that with time, revisional surgery or even mitralvalve replacement may be necessary.

Symptoms associated with severe MR with ARF whichevolved over a number of weeks or months usually respond topatient rest and diuretics. Indeed, it is our practice not to oper-ate acutely if symptoms are under control. If valve surgery isindicated because of LV chamber size or LV function, this canusually be deferred until the inflammatory markers, C-reactiveprotein (CRP) and erythrocyte sedimentation rate (ESR), havesubsided. Case 4, with a seven week history, represents a

Table 1 Clinical presentation and investigation

Case Age

(years)

Ethnicity Presenting

symptoms

Initial working

diagnosis

Examination

findings

ASOT/

anti-Dnase (IU)

ESR

(mm)

1 10 NZ

Maori

Cough, fever,

dyspnoea 2 days, blood

stained sputum 1 day

Pneumonia Tachycardic, tachypnoiec,

desaturation, hepatomegaly, PSM

223/1670

(13 days post

presentation)

–

2 10 Pacific

Island

Malaise 1 week, dyspnoea,

decreased LOC 1 day

(Down Syndrome)

Septic shock,

?paracetamol

overdose Infective

endocarditis

Hypothermic, hypoglycaemic,

bradypnoiec, unrecordable

saturations, GCS = 7/15, pulsatile

hepatomegaly, PSM, EDM

631/2360

(4 days post

presentation)

37

3 9 Cook

Island

Maori

Dyspnoea, cough, vomiting,

abdominal/sternal pain 3 days,

blood stained sputum,

decreased appetite 2 days

Acute hypoxia/

tachypnoea,

?pneumonia/cardiac

failure

Temperature = 36.2, tachypnoiec,

respiratory distress, desaturations,

displaced apex, apical PSM,

hepatomegaly

154/396

(3 days post

presentation)

74

4 6 NZ

Maori

Intermittent fever, decreased

appetite, vomiting, lethargy,

3 weeks, sore left knee/ankle

7 weeks, sore chest,

dyspnoea 3 days

ARF JVP = 4 cm, 4/6 apical PSM,

3/6 aortic diastolic murmur,

hepatomegaly, bibasal crepitations

803/1090

(day of

presentation)

97

Electrocardiography Chest X-ray (as interpreted by clinician) Echocardiogram

Sinus tachycardia, normal

LA & LV size

Density right lung, left lower lobe interstitial oedema.

Bilateral bronchopneumonia (Fig. 1)

ARF with ruptured chordae and flail posterior mitral valve leaflet.

Severe/torrential MR. Mild TR with RVSP 30 mmHg + right atrial

pressure. Mild LA and LV dilatation and normal LV function.

Normal LA & LV size Mild cardiomegaly, bilateral pleural effusion,

bilateral lower lobe consolidation

Severe AR with possible aortic root abscess. Severe MR with

possible anterior leaflet perforation. Mild TR. Mild PR.

Small lateral pericardial effusion. Moderate PHT.

Sinus tachycardia,

LA size increased

Marked ‘consolidation’ right lung and perihilar region

left lung due to bilateral pulmonary oedema (Fig. 2)

Torrential MR from a flail PMVL. Mild LA dilatation.

Hyperdynamic LV systolic function. Significant PHT.

Normal LA size

Mild LVH by voltage

Globular heart, moderately enlarged. No significant

pulmonary oedema or pleural fluid.

AMVL prolapse due to ruptured and elongated chord.

Severe MR. Moderate LA dilatation. Dilated LV with

good LV systolic function. Mild AR. Mild TR. PHT.

AMVL, anterior mitral valve leaflet; AR, aortic regurgitation; ARF, acute rheumatic fever; EDM, early diastolic murmur; GCS, Glasgow Coma Score; JVP, jugular

venous pressure; LOC, level of consciousness; LA, left atrium; LV, left ventricle; LVH, Left ventricular hypertrophy; MCL, mid-clavicular line; MR, mitral

regurgitation; PH/R, heart pulse rate; PHT, pulmonary hypertension; PMVL, posterior mitral valve leaflet; PSM, pan systolic murmur; PR, pulmonary

regurgitation; R/R, respiratory rate. RV, right ventricle; RVSP, right ventricular systolic pressure; TR, tricuspid regurgitation.

136 Journal of Paediatrics and Child Health 44 (2008) 134–137© 2007 The Authors

Journal compilation © 2007 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

Fulminant acute rheumatic fever Y Anderson et al.

scenario in between the fulminant life threatening pulmonaryoedema of cases 1–3 and the more typical ARF with severe MRprogressing over weeks. As this patient was in cardiorespiratoryfailure, operation could not be deferred. The operation of choicefor chronic rheumatic MR is valve repair.8 Lower operativemortality and better late outcome support efforts to repair thevalves acutely when patients are failing medical treatment.9,10

The role of corticosteroids or bed rest for continued rheumaticinflammation following valve surgery has not been studied norlikely to be, due to small numbers of patients and multipleconfounding factors.

It is difficult to estimate the incidence of chordal rupture orgross elongation resulting in severe sudden onset heart failurein ARF. We estimate this occurs in 1–3% of true first episodes

Table 2 Surgical management, and outcome

Initial management Surgical findings Day of surgery from admission Surgical management Histology

Ventilation,

inotropes

3 of 4 major chordae to PMVL ruptured Day 1. Mitral valve repair including insertion of artificial

chordae and 26 mm Carpentier Edward ring.

Uninterpretable as tissue

not fixed over weekend.

Ventilation,

inotropes

Pericarditis, inflamed AMVL,

AMVL stretched chordae

No vegetations

Day 5. Homograft aortic root replacement. Mitral valve

repair using 24 mm Carpentier ring.

Tricuspid annuloplasty.

Acute and chronic

inflammation.

Ventilated, inotropes Major chordae group to PMVL

ruptured with half flail leaflet

Day 3. Mitral valve repair. Annuloplasty ring not used. Acute inflammation with

some Aschoff bodies.

Diuretics, oxygen.

Monitored in

intensive care unit

Ruptured central chord AMVL, stretched

other AMVL chords, medial PMVL

prolapse with elongated chords

Day 3. Mitral valve repair using 24 mm Carpentier

Edwards ring and tricuspid annuloplasty.Leaflet had nodular

thickening, early

fibrosis.

Outcome of mitral repair in hospital Latest follow-up

Resolution of symptoms. Trivial MR. Mobilised early. MR returned over 2 months. MV replacement 11 months post MV repair. NYHA 1 on warfarin.

Resolution of symptoms, mild MR. Moderate MR at 8 months post MV repair. Asymptomatic.

Resolution of symptoms, mild MR. 22 months post MV repair. NYHA 1.

Resolution of symptoms, moderate MR, mobilised

cautiously.

Mitral valve replacement 10 months after repair for severe MR. NYHA 1 on warfarin.

AMVL, anterior mitral valve leaflet; AR, aortic regurgitation; LA, left atrium; LV, left ventricle; MR, mitral regurgitation; MV, mitral valve; NYHA, New York

Heart Association; PHT, pulmonary hypertension; PMVL, posterior mitral valve leaflet; PR, pulmonary regurgitation; RV, right ventricle; TR, tricuspid

regurgitation.

Fig. 1 Chest X-ray of case 1 on presentation showing bilateral pulmonary

oedema.

Fig. 2 Chest X-ray of case 3 on presentation showing dense pulmonary

oedema with dominant right lung involvement.

Y Anderson et al. Fulminant acute rheumatic fever

Journal of Paediatrics and Child Health 44 (2008) 134–137 137© 2007 The AuthorsJournal compilation © 2007 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

of ARF. This is based on the number of cases seen per year.Currently, between 100 and 150 new cases of ARF occur in NewZealand per year. Other evidence comes from the recentlyreported series from Utah12 as well postmortem series ofchildren dying of ARF. It is likely that there are still manychildren in developing countries who die acutely with this com-plication because it is not recognised or accurate echocardio-graphy is not available, or there is limited access to cardiacsurgical units.

Acknowledgements

We are grateful to Barbara Semb, Research Secretary,Green Lane Research and Education Fund for secretarialassistance.

References

1 Hwang W, Lam K. Rupture of chordae tendinae during acute rheumatic carditis. Br. Heart J. 1968; 30: 429–31.

2 De Moor M, Lachman P, Human D. Rupture of tendinous chords during acute rheumatic carditis in young children. Int. J. Cardiol. 1986; 12: 353–7.

3 Wu YN, Lue HC, Hou SH, How SW. Rupture of chordae tendinae in acute rheumatic carditis: report of one case. Chung-Hua Min Kuo Hsiao Erh Koi Hseuh Hui Tsa Chih 1992; 33: 376–82.

4 Haizlip J, Di Russo G, Vernon D, Tani L. Flail posterior leaflet of the mitral valve in acute rheumatic carditis. Pediatr. Cardiol. 2004; 25: 165–6.

5 El-Menyar A, Al-Hroob A, Numan M, Gendi S, Fawzy I. Unilateral pulmonary edema: unusual presentation of acute rheumatic fever. Pediatr. Cardiol. 2005; 26: 700–2.

6 Braunwald E. Valvular heart disease. In: Braunwald E, ed. Heart Disease, Vol. 2, 4th edn. Philadelphia: W.B. Saunders Company, 1992; 1026–7.

7 Mahajan C, Bidwai P, Walia B, Berry J. Some uncommon manifestations of rheumatic fever. Indian J. Pediatr. 1973; 40: 102–5.

8 Talwar S, Rajesh MR, Subramanian A, Saxena A, Kumar AS. Mitral valve repair in children with rheumatic heart disease. J. Thorac. Cardiovasc. Surg. 2005; 129: 875–9.

9 Chauvaud S, Fuzellier JF, Berrebi A, Deloche A, Fabiani JN, Carpentier A. Long-term (29 years) results of reconstructive surgery in rheumatic mitral valve insufficiency. Circulation 2001; 104 (12 Suppl. 1): I12–15.

10 Enriquez-Sarano M, Schaff HV, Orszulak TA, Tajik AJ, Bailey KR, Frye RL. Valve repair improves the outcome of surgery for mitral regurgitation. A multivariate analysis. Circulation 1995; 91: 1022–8.

11 Park Myung K. The Pediatric Cardiology Handbook, 3rd edn. St Louis: Mosby, An affiliate of Elsevier Science, 2003; 149–5.

12 Hillman ND, Tani LY, Veasy LG et al. Current status of surgery for rheumatic carditis in children. Ann. Thorac. Surg. 2004; 78: 1403–8.