© 2006 European Society of Cardiology
Acute management of pregnancy associated cardiomyopathy with cardiac resynchronisation therapy
Department of Cardiology, Glenfield Hospital Groby Road, Leicester, LE3 9QP, United Kingdom
* Corresponding author. Tel.: +44 116 2563922. E-mail address: dt.chin{at}ukgateway.net
Key Words: Heart failure Pregnancy associated cardiomyopathy Cardiac resynchronisation
Received May 24, 2006; Revised September 28, 2006; Accepted December 13, 2006
| 1. Background |
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Pregnancy associated or peri-partum cardiomyopathy is a rare maternal complication of gestation with an estimated incidence of between 1 per 3000 to 4000 live births [1,2]. Diagnostic criteria suggested by Demakis et al. [3] include: A: the development of cardiac failure in the last month of pregnancy or within 5 months of delivery; B: absence of an identifiable cause for the cardiac failure; C: absence of recognizable heart disease prior to the last month of pregnancy; and D: echocardiographic left ventricular systolic dysfunction. The aetiology remains unclear although recent studies have suggested a higher incidence in those mothers above 30 years of age and an association with gestational hypertension and twin pregnancies [4]. Despite significant improvements in the management of heart failure, the morbidity and mortality related to this condition remain significant, with mortality rates of between 9 and 32% and cardiac transplantation required in up to 10% of survivors [5,6]. We describe a case where acute, life-threatening haemodynamic deterioration was successfully managed with cardiac resynchronisation therapy.
| 2. Case report |
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A 28-year-old female was admitted to a district general hospital at 38 weeks of pregnancy with symptoms of orthopnoea, paroxysmal nocturnal dyspnoea, persistent cough and an exercise tolerance of 9 m. Initial examination revealed a blood pressure of 130/80 mmHg, a regular pulse of 140 bpm, an elevated jugular venous pressure (JVP) and inspiratory crepitations in the lung. A provisional diagnosis of left ventricular dysfunction was made and treatment was initiated with oral frusemide 40 mg o.d.
Initial blood results revealed a mild normocytic anaemia of 11.4 g/dl, otherwise urea and electrolytes, liver function tests and creatine kinase were all within normal limits. Chest X-ray showed cardiomegaly with right basal shadowing. ECG showed sinus tachycardia of 140 bpm, left bundle branch block pattern with a QRS duration of 160 ms.
Echocardiography revealed a dilated left ventricle with left ventricular internal diameter in diastole (LVIDd) of 6.3 cm with global hypokinesis and severely impaired left ventricular systolic function. There was 2D evidence of intra-ventricular dyssynchrony [7] with a septal to posterior wall motion delay of 310 ms [8], inter-ventricular dyssynchrony with an aortic pre-ejection period of 160 ms, pulmonary pre-ejection period of 30 ms giving a pre-ejection delay of 130 ms [9] and left sided AV dyssynchrony was demonstrated via diastolic filling time of 200 ms to R-R of 520 ms [10] (ratio 0.38) There was severe functional mitral regurgitation. The right ventricle was also mildly dilated with reduced contraction, the inferior vena cava was dilated with loss of collapse on inspiration, pulmonary artery pressure estimates were 38 mmHg (plus right atrial pressure), in keeping with moderate pulmonary hypertension. The patient was diagnosed with pregnancy associated cardiomyopathy and transferred to a centre providing both cardiology and obstetric services.
The same day the patient began to experience uterine contractions and obstetric advice was sought. The patient's cardiac state deteriorated developing atrial flutter with 2:1 block associated with a fall in blood pressure to 90/50 mmHg despite pharmacological therapy of beta-blocker, flecainide and amiodarone. DC cardioversion of the mother back to sinus rhythm was performed followed by emergency Caesarean section and transfer to a tertiary cardiology centre. Blood pressures remained low with mean arterial pressure around 75 mmHg and she required intra-aortic balloon pump and inotropic support with enoximone. Ventilatory support was maintained with intermittent positive pressure ventilation.
She was managed on the Intensive Care Unit and was weaned off ventilation 1-2 days post-Caesarean section. However, there was a great difficulty weaning from 1:1 balloon pump support providing augmented pressures of 95-105 mmHg and mean pressures of 75-85 mmHg despite the use of enoximone for inotropic support. Urine output and renal function were maintained, and PCWP was 20-25 mmHg. In view of the evidence of both mechanical and electrical dyssynchrony, consent was taken and a Guidant Contak Renewal TR2 CRT-P biventricular pacemaker was successfully inserted. Following insertion there was a rapid increase in augmented systolic blood pressure from 100 to 140 mmHg; the patient was successfully weaned from intra-aortic balloon pump within 3 h and discharged within 5 days receiving oral amiodarone, ACE inhibitor and beta blockade.
Echocardiography performed prior to discharge confirmed an improvement in ejection fraction from 25 to 32% [Fig. 1]. Follow-up with both 2D and 3D echocardiography at 1 month confirmed an LVEF of 32% and LVIDd of 4.8 cm [Fig. 2] and improvement in echo indices of dyssynchrony. During echo-guided device optimisation, the diastolic filling time/RR ratio improved to 0.45 and the left ventricular outflow tract velocity-time integral increased from 17.7 to 21.3 cm. Interrogation of the pacing device also demonstrated an improvement in heart rate variability foot-print area of around 300%, and an increase in SDANN of 20 ms. Heart failure diagnostics showed an increased activity from 6%/day to a plateau of 8-11%/day in keeping with the patient's improvement to NYHA II status.
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| 3. Discussion |
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Cardiac resynchronisation therapy benefits patients with chronic severe heart failure and cardiac dyssynchrony [11-13]. A recent study by Cleland et al. demonstrated an absolute risk reduction of a primary outcome of death or hospitalisation of 16% with cardiac resynchronisation plus optimal pharmacotherapy versus pharmacotherapy alone in patients with NYHA class III or IV heart failure (p<0.001) [14]. No studies to date have assessed the use of cardiac resynchronisation in the acutely unstable patient and current European guidelines only recommend use in chronic heart failure.
This case report demonstrates the use of cardiac resynchronisation in an acutely unstable patient with pregnancy associated cardiomyopathy. Although to date we are not aware of the long term outcome of this patient, we have demonstrated that this may be a potential treatment option in such patients. Recent studies have demonstrated that in 50% of such patients, cardiac function will improve spontaneously [3] and our treatment may well act as a "bridge" to such recovery. Our patient required intra-aortic balloon pump support which was discontinued after insertion of the biventricular pacemaker demonstrating the rapid action of such a device. This allowed an early move from the intensive care department allowing bonding with child and almost certainly an early discharge.
Procedural complications are likely to be increased in patients with acute heart failure and therefore further studies are required to fully assess the risk/benefit ratio in such a population.
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