Year : 2011 | Volume
: 1 | Issue : 1 | Page : 41--45
Anaesthetic management of two cases of peripartum cardiomyopathy
Bhawna Soni, PL Gautam, Anju Grewal, Harminder Kaur
Department of Anesthesiology, Dayanand Medical College and Hospital, Ludhiana, India
Professor, Department of Anesthesiology, Dayanand Medical College and Hospital, Ludhiana
Anaesthetic management for caesarean section of a patient with peripartum cardiomyopathy (PPCM) can be challenging. In this case report, we describe the anaesthetic management of two cases of PPCM posted for emergency caesarean section. Anaesthetic management was directed towards optimization of myocardial contractility, preload and after load. No adverse events or complications were observed.
|How to cite this article:|
Soni B, Gautam P L, Grewal A, Kaur H. Anaesthetic management of two cases of peripartum cardiomyopathy.J Obstet Anaesth Crit Care 2011;1:41-45
|How to cite this URL:|
Soni B, Gautam P L, Grewal A, Kaur H. Anaesthetic management of two cases of peripartum cardiomyopathy. J Obstet Anaesth Crit Care [serial online] 2011 [cited 2021 Jan 15 ];1:41-45
Available from: https://www.joacc.com/text.asp?2011/1/1/41/84256
Peripartum cardiomyopathy (PPCM) is an unusual form of dilated cardiomyopathy which manifests as acute heart failure in the last trimester of pregnancy or early postpartum period.  First recognized by Ritchie  in 19 th century, as an idiopathic disease, its diagnosis is based on exclusion of other identifiable causes. , Anaesthetic management of caesarean section in PPCM patients can be a challenge to the anesthesiologist. , The goals of anaesthetic management include maintenance of optimal ventricular preload and after load while avoiding any anaesthesia induced myocardial depression.  A vigilant monitoring both noninvasive and invasive is essential throughout the surgery and in the postoperative period to avoid complications like arrhythmias (atrial fibrillation), hypotension, hypoxemia, pulmonary edema, electrolyte disturbances, myocardial ischemia, thromboembolism, and even sudden death. ,,,,,
A 32-year-old female presented with signs and symptoms of acute onset congestive cardiac failure comprising mainly of breathlessness grade 4, persistent nocturnal cough, chest pain, jugular venous distension, systolic murmur, and bilateral basal crepitations in the third trimester at 7 th month of gestation. She reported regular antenatal checkups during the first and second trimesters with no previous history of preeclampsia or any other co-morbid condition. Her previous pregnancy four years back was uneventful.
An initial workup of the patient at this time revealed non-specific ST-T segment wave changes on electrocardiogram (ECG) and echocardiographic findings of poor ejection fraction (LVEF-15%), left ventricle diastolic dysfunction grade 3, dilated right ventricle and right atrium with mild mitral regurgitation (MR), suggesting PPCM. She was medically managed on digoxin and beta blocker (carvedilol) once daily, furosemide 40 mg, enoxaparin 40 mg twice daily along with oxygen therapy. She was discharged on request and put on medical therapy and daily oxygen therapy at home. She was advised continuous follow up and elective caesarean section near term.
However, she presented to the hospital at term in labor with rupture of membranes along with shortness of breath, orthopnea, chest pain, and peripheral edema. Physical examination revealed jugular venous distension, peripheral edema, loud S3, systolic murmur, and bilateral basal crepitations. An echocardiography repeated at this time revealed LVEF of 20% with global hypokinesia, severe MR with mild tricuspid regurgitation (TR) and pulmonary artery (PA) pressures 35 mmHg and dilated right atrial and ventricle chambers. The obstetrician planned an urgent caesarean section in view of a non-reassuring fetal heart rate, previous leaking due to spontaneous ruptures of membranes and maternal decompensation.
Preoperatively baseline pulse rate was 120 beats/min, regular with non-invasive blood pressure (NIBP) of 110/70 mmHg. A written informed consent was taken after discussing the risks and prognosis with the mother and family. Routine laboratory investigations were within normal range. She was wheeled into the operation theater in left lateral position and on continuous oxygen therapy. General anaesthesia was planned in view of the emergency nature of the surgery, presence of anticoagulation, and a low ejection fraction with severe left ventricle systolic impairment. All standard monitoring was initiated in the form of heart rate, NIBP, and SpO2 with the table tilted to left lateral position. An 18-guage peripheral IV cannula was inserted and left radial artery was cannulated under local anaesthesia (LA). Central right central venous cannulation was done under LA and a pulmonary artery (PA) catheter was inserted and PA pressures were monitored. The baseline PA pressures were 65/10, heart rate was around 100 beats/min and the invasive arterial blood pressure (IABP) was 118/76 mm of Hg. After preoxygenation with 100% oxygen for 3 mins, anaesthesia was induced with titrated doses of morphine 6 mg i.v and thiopentone 150 mg till abolition of eyelash reflex. After confirming adequate chest rise with bag mask ventilation, endotracheal intubation using a 7.5 mm cuffed PVC endotracheal tube (ETT) was facilitated with a neuromuscular blocking agent, inj atracurium given 30 mg i.v. slowly. The position of the ETT was confirmed by auscultation and EtCO2 graph and the ETT was secured. After anaesthesia induction, IABP fell to 86/60 mm of Hg. Dopamine infusion was started at a dose of 2-5 microgm/kg/min and titrated according to blood pressure. Hemodynamic parameters remained stable thereafter with an IABP of around 95 to 100 mmHg systolic and PA pressures between 65/10 to 45/10 with wide pulse pressures. Twenty ml saline boluses were given intermittently to maintain adequate preload. Anaesthesia was maintained with intermittent titrated use of isoflurane (0.2%) in oxygen and air and oxygen saturation was maintained between 97-99%. After the delivery of the baby, an infusion of oxytocin was started with 20 units in 500 ml of normal saline. The child was healthy, weighing 2.1 kg with an appearance, pulse, grimace, activity, and respiration (APGAR) score of 8-9 at the time of birth. PA pressures were maintained around 35 to 30. Occasional premature ventricular contractions were noted on the ECG monitor. Arterial blood gas analysis was within normal limits, but the potassium was 3.2 meq/l; hence potassium supplementation was started. At the end of the procedure, patient was kept electively intubated and shifted to ICU on ventilatory support. Postoperatively, dopamine infusion was gradually tapered 24 hrs later. She was weaned off the ventilator and trachea extubated 6 hrs later uneventfully. Post extubation, she was conscious, oriented, and maintaining SpO 2 of 97% on ventimask at FiO 2 0.5 and IABP of 100/65 mm of Hg. PA pressures were maintained around 30-35/10 - 14. She was discharged to the ward on the 6 th day with stable hemodynamics, put on diuretics, digoxin and beta-blockers, and is now on regular follow-up after discharge from the hospital.
A 37-year-old obese (BMI 30), multigravida parturient was referred to our hospital emergency at 39 weeks of pregnancy in labor, fetal distress, and features suggestive of acute congestive cardiac failure. She was a previously diagnosed case of peripartum cardiomyopathy (PPCM) during the seventh month of gestation. She was admitted to a cardiac unit of a local hospital during the 7 th month with acute congestive heart failure and a diagnosis of PPCM was made based on clinical and echocardiography findings. The previous two trimesters were uneventful. There was no history of preeclampsia or any other comorbid condition. She was receiving diuretics, digoxin, low molecular weight heparin (LMWH), and beta blockers daily. Echocardiography done at 7 th month of gestation had revealed dilated cardiac chambers with a left ventricular ejection fraction of 20%. At this time, she was in labor, had cough, orthopnea, nocturnal dyspnoea, and chest pain. Physical examination revealed pallor, pedal edema, systolic murmur, heart rate of 130 beats/min with sinus rhythm, NIBP of 100/70 mmHg, respiratory rate of 35/min along with use of accessory muscles of respiration with fine basal crepitations bilaterally. She was maintaining saturation of 90-92% on high flow oxygen mask as her oxygen saturation on room air was 85%. Though routine laboratory investigation including renal function tests and coagulation profile were within normal range, her hemoglobin was 9 gm% and ECG showed ventricular ectopics < 5/min. A repeat echocardiography showed dilated chambers with LVEF 25% and global hypokinesia. Arterial blood gas (ABG) showed pH of 7.3, pO 2 of 77 mmHg, pCO 2 of 35 mmHg, and HCO 3 30 mmol/l. It was planned to administer general anaesthesia to this patient in view of ongoing fetal distress; and an informed consent was obtained. She was shifted to operation theater (OT) with a wedge under her right hip and standard monitoring was instituted. After insertion of a 14 guage iv cannula on the dorsum of right hand and a left radial arterial cannula, rapid sequence anaesthesia induction was carried out with slow titrating doses of inj. fentanyl 100 microgm, inj thiopentone 100 mg, and inj suxamethonium 100 mg and a 7.5 mm ETT inserted and secured after confirming correct placement. Central venous cannulation was inserted postinduction as the patient was distressed and found lying supine flat uncomfortable. After delivery of baby (Apgar 8 at 1 min), slow syntocinon infusion with 20 units in 500 ml of normal saline was commenced. Central venous pressure (CVP) was 22 cms of Normal Saline (NS), hence 40 mg furosemide i.v. was given. Anaesthesia was maintained with isoflurane (0.2-0.6%) in oxygen and air and oxygen saturation was maintained between 93 and 97%. Dopamine infusion was titrated in doses of 5-10 microgm/kg/min to maintain stable hemodynamics which was gradually tapered off postoperatively over two days. At the end of surgery, heart rate (HR) was 106 beats/min and BP was 110/70 mmHg. Positive end-expiratory pressure (PEEP) of 8 cm was added to maintain a saturation of around 95-97%. Patient was kept on elective ventilatory support in ICU for two days and then weaned off the ventilator gradually after two days and discharged from ICU after 5 days. Postoperative investigations including serum electrolytes and ABG were within normal limits. She was managed with antibioitics, LMWH, digoxin, loop diuretics, and beta blockers and discharged from hospital after 10 days.
PPCM is characterized with onset of acute congestive heart failure without any demonstrable cause in the last trimester of pregnancy or within the first 6 months after delivery. , The incidence of PPCM is reported as 1:300 to 1:15000 in the western literature; however, incidence in Indian scenario is lacking due to paucity of data. ,
Due to its association with higher morbidity and mortality (30-60%), , various Western countries enroll such cases in their high risk obstetric anaesthesia registries, wherein details of anaesthetic management of these cases is also registered.  We, in India, also need to form such registries that would help us develop our database for future directions on optimal management and prognosis of PPCM.
The diagnostic criteria for PPCM are (1) the development of congestive heart failure secondary to decreased left ventricular systolic function in the last month of pregnancy or within five months postpartum, (2) absence of pre-existing cardiac dysfunction, (3) absence of determinable cause of cardiomyopathy and more recently (4) left ventricular systolic dysfunction demonstrated by classic echocardiographic criteria: ejection fraction less than 45% or M-mode fractional shortening less than 30% or both, and end-diastolic dimension more than 2.7 cm/m 2.,,,, Echocardiographically demonstrable improvement in left ventricle function has been added as 4 th criteria, recently.  Establishing a specific cause for left ventricle failure excludes the diagnosis of PPCM  It is usually a diagnosis of exclusion, as one needs to exclude differential diagnoses like other types of cardiomyopathies, valvulopathies, pulmonary embolism, infections, metabolic or ischemic causes of myocardial dysfunction which are amenable to therapy. ,,
Despite clearly outlined criteria, the diagnosis of PPCM presents a challenge because many normal parturients in the last month of pregnancy experience dyspnoea, fatigue, and pedal oedema and moreover the initial symptoms could mimic mild upper respiratory tract infection.  A high index of suspicion of heart failure should be kept in mind for symptoms like paroxysmal noctural dyspnoea, orthopnea, chest pain, persistent cough, new regurgitant murmurs, pulmonary crackles or crepts, elevated jugular venous pressure, hepatomegaly, and postural hypotension reflecting low cardiac output. ,, Both our patients had features like chest pain, systolic murmurs, dyspnoea, and elevated jugular pressure which suggested acute congestive heart failure. Final diagnosis was clinched by the echocardiographic report of low LVEF with global hypokinesia and dilatation of all cardiac chambers.
The etiology of PPCM remains elusive, however, viral, autoimmune, hemodynamic stress of pregnancy, cytokine-mediated inflammation, Gq-related myocyte apoptosis, oxidative stress-induced Cathepsin D production, selenium deficiency, and idiopathic causes are being hypothesized. ,,,, Other possible etiologies include nutritional deficiencies, small-vessel coronary artery disease, excessive salt intake, and peripartum fluid shifts.  Maternal age more than 30 years, multiparity, eclampsia, obesity, racial origin (African descent), hypertension, malnutrition, and prolonged tocolysis are predisposing factors. ,,,,,,,
Our patients had underlying risk factors such as multiparty, advanced maternal age, obesity (second case) with clinical features of acute congestive cardiac failure and echocardiographic changes consistent with PPCM.
Management is mainly symptomatic and aims to reduce after load and preload and enhance myocardial contractility, reduce arrythmogenicity; hence, includes bed rest, digitalis, loop diuretics, vasodilators, β-blockers, anticoagulants, and ionotropic support.,,,,,, If supportive treatment fails, cardiac transplantation may be indicated. ,,, Early delivery is the major consideration to avoid deleterious effects on parturient either by instrumental vaginal or caesarean section. , Close liaison with the patient's cardiologist, intensivist, and obstetrician is essential.
Anaesthetic management for caesarean delivery in women with PPCM should incorporate measures to optimally reduce preload and after load while maintaining an increase in myocardial contractility. ,,,,,,, Correct choice of anaesthesia and precise titration is crucial for a favorable outcome.  Various case reports describe both general anaesthesia and regional anaesthesia techniques like combined spinal epidural anaesthesia (CSE), sequential CSE anaesthesia or epidural anaesthesia or continuous spinal anaesthesia. ,,,,,,2,,, The challenge in either of these techniques is to avoid myocardial depression, hypovolemia, and prevent any increase in pre- and after load. , The use of invasive monitoring is also recommended to guide anaesthetic management, though successful outcomes have been reported with non-invasive monitoring as well.  Anticoagulation is indicated as PPCM increases the risk of thromboembolic events. ,
We opted for general anaesthesia (GA), ,,, guided with invasive monitoring due to the urgent nature of surgery, presence of anticoagulation and severity of PPCM. Though performing a rapid sequence induction on a patient with compromised cardiac function can be very challenging, ,, a smooth titrated induction and maintenance of anaesthesia obviated the stress of laryngoscopy and intubation and diminished the sympathetic responses. Hemodynamic stabilization was achieved by invasive monitoring which guided fluid therapy and ionotropic infusions. Avoidance of high concentrations of volatile agents, other drugs like atropine, ephedrine, etc and hypovolemia prevented dramatic cardiac depression and uncontrolled changes in after load and preload. ,,, As boluses of oxytocin can cause hypotension and tachycardia, we used infusion of oxytocin. , We also avoided the stress of extubation by planning elective ventilation postoperatively followed by gradual weaning and extubation under full invasive monitoring. In a case report,  remifentanil and etomidate have been found to maintain hemodynamic stability while avoiding myocardial depression; however, we could not use these agents due to non-availability in our set-up.
In elective conditions, a carefully administered regional anaesthetic technique like CSE or sequential epidural has been found to be beneficial as it avoids the stress of GA and produces changes in preload and after load which mimic the goals of hemodynamic variability in these patients thereby improving myocardial performance by reducing LV after load. ,,,,,,,, However, a subarachnoid block can induce catastrophic hemodynamic instability secondary to reduction in systemic vascular resistance (SVR) and limited cardiac reserve. 
In the course of the disease, cardiac function recovers in around half the women with PPCM, but in those with persistent left ventricular dysfunction even after 6-12 months, a mortality of up to 85% has been reported, indicating irreversibility and is an absolute contraindication for future pregnancies.  Those with documented poor recovery might be considered for cardiac transplants. 
PPCM is a rare disease of unknown cause that affects women in the child bearing years. The diagnosis of PPCM is challenging and that of exclusion which requires a high index of suspicion. Choice of anaesthesia needs to be guided based on the urgency of lower segment caesarean section (LSCS) and severity of PPCM. The primary anaesthetic goal should be to avoid radical swings in preload and after load and avoidance of myocardial depression, while alleviating the symptoms of congestive heart failure.
|1||Abboud J, Murad Y, Chen-Scarabelli C, Saravolatz L, Scarabelli TM. Peripartum cardiomyopathy: A comprehensive review. Int J Cardiol 2007;118:295-303.|
|2||Ritchie C. Clinical contribution to the patho-diagnosis and treatment of certain chronic diseases of the heart. Edinb Med J 1850;2:2.|
|3||Zangrillo A, Landoni G, Pappalardo F, Oppizzi M, Torri G. Different anesthesiological management in two high risk pregnant women with heart failure undergoing emergency caesarean section. Minerva Anestesiol 2005;71:227-36.|
|4||Bhakta P, Biswas BK, Banerjee B. Peripartum cardiomyopathy: Review of the literature. Yonsei Med J 2007;48:731-47.|
|5||Heider AL, Kuller JA, Strauss RA, Wells SR. Peripartum cardiomyopathy: A review of the literature. Obstet Gynecol Surv 1999;54:526-31.|
|6||Kotekar N, Nagalakshmi NV, Chandrashekar. A rare case of peripartum cardiomyopathy posted for caesarean section. Indian J Anaesth 2007;51:60-4.|
|7||McIndoe AK, Hammond EJ, Babington PC. Peripartum cardiomyopathy presenting as a cardiac arrest at induction of anaesthesia for emergency caesarean section. Br J Anaesth 1995;75:97-101.|
|8||Bhakta P, Mishra P, Bakshi A, Langer V. Case report and mini literature review: anaesthetic management for severe peripartum cardiomyopathy complicated with preeclampsia using sufetanil in combined spinal epidural anaesthesia. Yonsei Med J 2011;52:1-12.|
|9||Report of the WHO/ISFC task force on the definition and classification of cardiomyopathies. British Heart J 1980;44:672-3. |
|10||Hibbard JU, Lindheimer M, Lang RM. A modified definition for peripartum cardiomyopathy and prognosis based on echocardiography. Obstet Gynecol 1999:94;311-6.|
|11||Demakis JG, Rahimtoola SH. Peripartum cardiomyopathy. Circulation 1971;44:964-8.|
|12||Veille JC. Peripartum eardiomyopathies: a review. Am J Obstet Gynecol 1984;148:805-18.|
|13||Homans DC. Peripartum cardiomyopathy: current concepts. N Engl J Med 1985;312:1432-7.|
|14||Lewis NL, Dob DP, Yentis SM. UK registry of high-risk obstetric anaesthesia: Arrhythmias, cardiomyopathy, aortic stenosis, transposition of the great arteries and Marfan's syndrome. Int J Obstet Anesth 2003;12:28-34.|
|15||Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, et al. Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review. JAMA 2000;283:1183-8.|
|16||Chapa JB, Heiberger HB, Weinert L, Decara J, Lang RM, Hibbard JU. Prognostic value of echocardiography in peripartum cardiomyopathy. Obstet Gynecol 2005;105:1303-8.|
|17||Witlin AG, Mabie WC, Sibai BM. Peripartum cardiomyopathy: A longitudinal echocardiographic study. Am J Obstet Gynecol 1997;177:1129-32.|
|18||Lampert M, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995;130:860-70.|
|19||Shnaider R, Ezri T, Szmuk P, Larson S, Warters RD, Katz J. Combined spinal-epidural anaesthesia for caesarean section in a patient with peripartum dilated cardiomyopathy. Can J Anaesth 2001;48:681-3.|
|20||Indira K, Sanjeev K, Sunanda G. Sequential combined spinal epidural anaesthesia for caesarean section in peripartum cardiomyopathy. Indian J Anaesth 2007;51:137.|
|21||Lampert MB, Hibbard J, Weinert L, Briller J, Lindheimer M, Lang RM. Peripartum heart failure associated with prolonged tocolytic therapy. Am J Obstet Gynecol 1993;168:493-5.|
|22||O'Connell JB, Costanzo-Nordin MR, Subramanian R, Robinson JA, Wallis DE, Scanlon PJ, et al. Peripartum cardiomyopathy: Clinical, hemodynamic, histologic and prognostic characteristics. J Am Coll Cardiol 1986;8:52-6.|
|23||Fett JD, Ansari AA, Sundstrom JB, Combs GF. Peripartum cardiomyopathy: A selenium disconnection and an autoimmune connection. Int J Cardiol 2002;86:311-6. |
|24||Sanderson JE, Adesanya CO, Anjorin FI, Parry EH. Postpartum cardiac failure-heart failure due to volume overload? Am Heart J 1979;97:613-21.|
|25||Ntusi NB, Mayosi BM. Aetiology and risk factors of peripartum cardiomyopathy: A systemic review. Int J Cardiol 2009;131:168-79.|
|26||Pryn A, Bryden F, Reeve W, Young S, Patrick A, McGrady EM. Cardiomyopathy in pregnancy and caesarean section: four case reports. Int J Obstet Anesth 2007;16:68-73.|
|27||Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy. Lancet 2006;368:687-93.|
|28||Brown CS, Bertolet BD. Peripartum cardiomyopathy: a comprehensive review. Am J Obstet Gynecol 1998;178:409-14.|
|29||Malinow AM, Butterworth JF 4th, Johnson MD, Safon L, Rein M, Hartwell B, et al. Postpartum cardiomyopathy at caesarean delivery. Anesthesiology 1985;63:545-7.|
|30||Davidson NM, Parry EH. Peri-partum cardiac failure. Q J Med 1978;47:431-61. |
|31||George LM, Gatt SP, Lowe S. Peripartum cardiomyopathy: Four case histories and a commentary on anaesthetic management. Anaesth Intensive Care 1997;25:292-6.|
|32||Carlson KM, Browning JE, Eggleston MK, Gherman RB. Peripartum cardiomyopathy presenting as lower extremity arterial thromboembolism. A case report J Reprod Med 2000;45:351-3. |
|33||Futterman LG, Lemberg L: Peripartum cardiomyopathy: an ominous complication of pregnancy. Am J Crit Care 2000;9:362-6. |
|34||McCarroll CP, Paxton LD, Elliott P, Wilson DB. Use of remifentanil in a patient with peripartum cardiomyopathy requiring caesarean section. Br J Anaesth 2001;86:135-8. |
|35||Velickovic IA, Leicht CH. Continuous spinal anaesthesia for caesarean section in a parturient with severe recurrent peripartum cardiomyopathy. Int J Obstet Anesth 2004;13:40-3.|
|36||Gambling DR, Flanagan ML, Huckell VF, Lucas SB, Kim JH. Anaesthetic management and non-invasive monitoring for caesarean section in a patient with cardiomyopathy. Can J Anaesth 1987;34:505-8.|
|37||Kaufman I, Bondy R, Benjamin A. Peripartum cardiomyopathy and thromboembolism; anaesthetic management and clinical course of an obese, diabetic patient. Can J Anaesth 2003;50:161-5.|
|38||Bilehjani E, Kianfar AA, Toofan M, Fakhari S. Anaesthesia with etomidate and remifentanil for caesarean section in a patient with severe peripartum cardiomyopathy-A case report. Middle East J Anesthesiol 2008;19:1141-9.|