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An unusual cause of cardiac failure
  1. Nicholas R Balcombe
  1. Department of Health Care for the Elderly, Queens Hospital, Burton Hospitals NHS Trust, Belverdere Road, Burton-upon-Trent, Staffordshire DE13 0RB, UK

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    A 91-year-old woman, with long-standing swelling of both ankles, presented with a 2-month history of increasing swelling of both legs and lower abdomen. She was otherwise asymptomatic. She had undergone a mastectomy for carcinoma of the breast in 1981. At this time, no cardiac murmurs were documented and her electrocardiogram (ECG) showed normal sinus rhythm with a partial right bundle branch block. She remained well at follow-up. There was no history of ischaemic heart disease. Recently her general practitioner had commenced her on digoxin and diuretics for atrial fibrillation and the oedema.

    On examination she was acyanotic. Her apical heart rate was irregularly irregular with a ventricular rate of 70 beats/min, blood pressure was 140/80 mmHg and carotid pulses were small volume. The right ventricular impulse was prominent and there was evidence of right ventricular failure (the jugular venous pulse was raised 10 cm above the sternal angle, a smooth, regular, non-tender, liver was palpated three finger breadths below the right costal margin and there was bilateral, non-tender, pitting oedema of both legs up to and involving the lower abdomen). There was no ascites. On auscultation there was fixed splitting of the second heart sound but no increase in the pulmonary component. There was evidence of tricuspid regurgitation (a pansystolic murmur was heard at the left sternal edge in the fourth intercostal space, loudest during inspiration, with a pulsatile hepatomegaly and a large V wave in the jugular venous pulse). There was also evidence of increased blood flow across the pulmonary valve (an ejection systolic murmur was heard at the upper left sternal edge, loudest during inspiration).

    An ECG showed atrial fibrillation, right bundle branch block, right axis deviation and ventricular ectopic beats with coupling. A chest X-ray showed enlargement of both atria and the right ventricle. The pulmonary artery was dilated, with pulmonary plethora, and upper lobe venous distension. There were small bilateral basal pleural effusions.

    Questions

    1
    What investigation would you perform next?
    2
    What is the diagnosis?

    Answers

    QUESTION 1

    The patient presented with a clinical picture suggestive of cardiac failure (predominantly right sided) secondary to an atrial septal defect. The next investigation that should be performed is a transthoracic Doppler 2D echocardiogram, to allow identification of the atrial septal defect and direction of blood flow across the defect. It will also allow for assessment of ventricular function and confirmation of the valvular lesions.

    QUESTION 2

    An echocardiogram was performed and confirmed the presence of an ASD secundum, 2.1 cm in length with left to right shunting. Both atria and right ventricle were enlarged. Biventricular function was vigorous (ejection fraction 71%) and there was severe tricuspid regurgitation. The patient refused cardiac catheterisation and was treated with diuretics and angiotensin-converting enzyme inhibitors. Her digoxin was temporarily stopped in view of the ventricular ectopics and coupling, but was restarted once digoxin toxicity had been excluded. At the present time she remains well.

    Discussion

    Atrial septal defects are thought to shorten life expectancy. With increasing age, clinical deterioration occurs with the onset of atrial fibrillation, embolic phenomena, pulmonary hypertension, cardiac failure and bronchopulmonary infections, so that by middle age most patients are symptomatic.1 2

    Mortality figures suggest that in patients with clinically overt disease, 75% are dead by the age of 50 years and 90% by 60 years.3 Only two previous reports of survival into the ninth decade have been recorded.4 5 Our patient is unusual not only for her longevity, but also for her freedom from significant symptoms for so long.

    It is accepted that surgical closure of atrial septal defects in children and young adults carries a low operative mortality and should be performed to prevent future complications. The benefit of surgery in older patients remains debatable. Early studies claimed to show improvements in morbidity and mortality in adult patients treated surgically,1 but recent studies have found similar rates of morbidity and mortality in both medically and surgically treated adult patients, with survival of up to 91% in medically treated patients.2

    Clinical features of ostium secundum atrial septal defects

    Symptoms

    • rare in infancy

    • uncommon in childhood

    • usual in adults

    • palpitations

    • exertional dyspnoea

    • ankle swelling

    • chest pain

    • respiratory tract infection

      Signs

    • small volume pulse

    • atrial fibrillation

    • raised jugular venous pressure

    • peripheral oedema

    • ascites

    • prominent right ventricular impulse

    • loud first heart sound

    • loud P2

    • fourth heart sound

    • fixed splitting of second heart sound

    • pulmonary / tricuspid flow murmur

    • pulmonary / tricuspid regurgitant murmur

      Investigations

    • ECG

    • right bundle branch block

    • right axis deviation

    • atrial fibrillation

    • chest X-ray

    • right ventricular dilatation

    • right atrial enlargement

    • bi-atrial enlargement with atrial fibrillation

    • small aortic knuckle

    • pulmonary artery dilatation

    • pulmonary plethora

    This case illustrates that not all cases of cardiac failure in the elderly are due to common aetiologies, such as hypertension or ischaemia and, therefore, thorough evaluation of such cases is important, with echocardiogram forming an important part of that evaluation. The longevity and well being of our patient and of others of such longevity,4 5 also indicates that surgical intervention in middle-aged and elderly patients with atrial septal defects remains of questionable value.

    Final diagnosis

    Cardiac failure secondary to ostium secundum atrial septal defect.

    References

    View Abstract

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