Printer Friendly

High output cardiac failure in a parturient with hereditary haemorrhagic telangiectasia.

Hereditary haemorrhagic telangiectasia (HHT), or Osler-Rendu-Weber disease, is an autosomal dominant genetic condition that affects one in 5000 to 8000 (1). Although most literature on HHT and pregnancy deals primarily with pulmonary arteriovenous malformations (AVM), which cause significant morbidity and mortality (2-6), HHT affects the liver in 41 to 78% of cases (7). Most patients are asymptomatic, especially prior to pregnancy, but with the added oxygen demands of pregnancy they may present with high output cardiac failure, or less commonly with portal hypertension, biliary disease, abnormal liver function tests, encephalopathy and parenchymal abnormalities. We describe the case of a woman with high output cardiac failure in the presence of hepatic AVMs. Consent was obtained from the patient to write this case report.


A 27-year-old primigravid woman (80 kg, 165 cm) presented to hospital at 36 weeks gestation with worsening dyspnoea. She had a history of HHT, which was diagnosed after an episode of renal and gastrointestinal haemorrhage following mild trauma at 12 years of age. There was a strong family history, her mother having received liver transplantation for hepatic failure secondary to hepatic AVMs and her sister known to have cerebral AVMs.

Our patient was known to have hepatic AVMs throughout both lobes of the liver. Despite occasional mild gastrointestinal bleeding, a recent gastroscopy and colonoscopy had been normal. Computed tomography pulmonary angiogram did not demonstrate pulmonary vascular malformations and a cerebral magnetic resonance imaging scan did not show intracranial vascular malformations. She regularly consulted a hepatologist who monitored the size of her AVMs. She had asthma which was controlled with budesonide and salbutamol and she smoked three cigarettes a day. Prior to her first pregnancy she had normal exercise tolerance (New York Heart Association functional class one) and she managed a farm in a rural town.

The early stages of the pregnancy were uneventful. At 16 weeks gestation she developed exertional dyspnoea. On examination then she had a loud pulmonary second heart sound and a pulmonary flow murmur. A transthoracic echocardiogram (TTE) showed pulmonary hypertension (estimated pulmonary arterial pressure [PAP] 46 mmHg+right atrial pressure [RAP]) with an elevated cardiac output. The right and left ventricles were of normal size and function. She was referred to a cardiologist, who thought that the pulmonary hypertension reflected the increased cardiac output due to her hepatic AVMs. Repeat TTEs at 24 and 33 weeks gestation showed little change in the pulmonary pressures and her cardiac output was estimated as approximately 11 l/minute. The obstetrician planned with her a vaginal delivery at term.

The patient represented emergently at 36 weeks gestation with dyspnoea at rest. Her heart rate was 100 bpm, systemic blood pressure was 175/40 mmHg and oxygen saturation was 96% in room air. She was grossly orthopnoeic. Her renal function was normal and her urinalysis was negative for protein and blood. A TTE showed that the cardiac output had risen to 17 l/minute, with PAP of 34 mmHg+RAP. In consultation with her cardiologist, obstetrician and anaesthetist, it was decided to proceed with an urgent caesarean section because of the worsening maternal high output cardiac failure. In the meantime, she was given oxygen therapy and nebulised salbutamol as it was thought that her asthma may have contributed to the dyspnoea. Her liver function tests were normal except for a mild elevation in alkaline phosphatase levels.

The caesarean section was performed at an adult tertiary hospital, co-located with the tertiary referral obstetric hospital, as a combined case with consultant cardiac and obstetric anaesthetists, because of the potential complications of worsening cardiac failure requiring inotropic and ventilator support and the need for postoperative intensive care management. Due to the patient's cardiac and respiratory compromise a general anaesthetic was chosen. Her airway was predicted to be easy to intubate because of a Mallampati class one airway examination, full mouth-opening and a full range of neck flexion and extension. The patient was stabilised and fasted for seven hours and received ranitidine 150 mg orally for aspiration prophylaxis. Two large bore intravenous cannulae and a radial arterial cannula were inserted. The patient was placed in a semi-upright left lateral tilt position and after pre-oxygenation she was induced using fentanyl 1000 [micro]g, midazolam 5 mg and suxamethonium 150 mg. The laryngoscopic view was Grade 1 and an endotracheal tube was inserted rapidly, with cricoid pressure applied. Anaesthesia was maintained with isoflurane (end-tidal concentration 0.5%) and propofol (200 mg/h)

and the bispectral index monitor was used to monitor depth of anaesthesia. Muscle relaxation was maintained with pancuronium. Two boluses of 0.5 mg metaraminol were required to maintain mean arterial pressure within 20% of baseline. A pulmonary artery catheter (PAC) was inserted in the right internal jugular vein under ultrasound guidance. After the delivery of the baby, oxytocin 10 IU was administered in small incremental doses, an infusion commenced at 10 IU/hour and intravenous antibiotic given. The PAP after the delivery of the baby was 42/20 mmHg and cardiac output was 15.4 l/min. She had a total of 400 ml blood loss and one litre of crystalloid infused intravenously.

A live female baby was delivered, with Apgar scores of four and nine at one and five minutes respectively. The baby required resuscitation with continuous positive airway pressure ventilation via a facemask and 300 [micro]g of naloxone. She was transferred to the special care nursery for overnight observations and was subsequently discharged to the postnatal ward.

Postoperatively the patient was transferred to the adult intensive care unit (ICU), still intubated and ventilated. Her cardiac output measurements ranged from 12.9 to 18.8 l/minute. A chest X-ray showed signs consistent with pulmonary congestion, which was treated with fluid restriction and diuretics. Pain was treated with a combination of paracetamol and intravenous morphine. There was rapid symptomatic improvement and she was extubated within 24 hours and discharged home on day 11. A repeat TTE four weeks postpartum showed cardiac output of 8.6 l/minute and PAP 35 mmHg+RAP. She took seven months to recover to New York Heart Association functional class 2.

The patient's second pregnancy occurred 15 months after the birth of her first child, following lengthy discussions with a perinatal physician and geneticist. She was monitored closely by her obstetrician, cardiologist and hepatologist. Echocardiography at eight weeks gestation showed her cardiac output was 9.6 l/minute and PAP 24 mmHg+RAP. In the mid-second trimester she developed dyspnoea on exertion progressing to dyspnoea at rest at 24 weeks gestation. TTE at 24 weeks revealed a mildly dilated left ventricle with hyperdynamic systolic function (Figure 1). There was moderate biatrial enlargement and PAP was estimated as 40 mmHg+RAP (Figure 2, 3).

She underwent initial stabilisation with bed rest, diuretics and fluid restriction in the obstetric tertiary referral hospital and was then transferred to the adjacent adult tertiary referral hospital where she was supervised by the obstetric team for regular foetal surveillance, the cardiology team for titration of therapy for cardiac failure, and the anaesthetic team regarding method of and timing of delivery. Her liver function tests remained normal. Due to the early and rapid progression of her cardiac failure, she remained an inpatient from the 24th week with a plan to deliver the baby via caesarean section with a general anaesthetic at 30 weeks gestation. This gestation was chosen to balance the foetal risks with those of her worsening cardiac failure. General anaesthesia was chosen based on her previous successful general anaesthetic with easy intubation and to facilitate control of potential respiratory or cardiac complications.



General anaesthesia for caesarean section and tubal ligation was performed by consultant obstetric and cardiac anaesthetists. Prior to induction a radial arterial catheter and a right internal jugular vein pulmonary artery catheter was inserted. Her baseline blood pressure was 125/40, heart rate 90 bpm and oxygen saturation 98%. She received fentanyl 500 [micro]g, a titrated dose of propofol 140 mg and suxamethonium 100 mg. Cricoid pressure was applied following loss of consciousness and the trachea was secured rapidly. A small dose of atracurium was administered and anaesthesia was maintained with sevoflurane using bispectral index monitoring. An intraoperative transoesophageal echocardiogram (TOE) showed a hyperdynamic circulation (cardiac output 19 l/minute, closely correlated with that measured via thermodilution). She remained haemodynamically stable, without the need for vasopressors or inotropes. A live female baby was delivered with Apgar scores of five and eight at one and five minutes respectively. The baby was intubated for respiratory distress and transferred to the neonatal ICU. Manual rubbing of the uterine fundus achieved excellent uterine contraction and a Syntocinon infusion was commenced at 10 IU/hour. Approximately 750 ml of crystalloid solution was given intraoperatively and the estimated blood loss was 400 ml. The patient was extubated alert and comfortable at the end of the surgery and transferred to the ICU for overnight monitoring. Postoperative analgesia was achieved with morphine patient-controlled analgesia and paracetamol. The cardiac output measured in ICU was 15.3 l/minute. She made a rapid recovery and was discharged home four days after her surgery. At her six months review she was New York Heart Association functional level 2 and enjoying life with her two children.



This case highlights the issues involved in managing a pregnant woman with predominantly hepatic HHT and cardiac failure and the importance of a multidisciplinary team approach (9). When not pregnant, treatment of hepatic AVMs depends on the presentation and severity (10). Milder grades do not require treatment. High output cardiac failure is treated medically and portal hypertension both medically and endoscopically. Various surgical approaches have been suggested for treatment-resistant hepatic AVMs, including surgical ligation or embolisation of the hepatic artery, although fatal hepatic necrosis after embolisation has been reported (11). Ultimately, orthotopic liver transplantation may be required for severe hepatic AVMs (12,13). In pregnancy there is less published experience with managing these patients and most literature deals with patients with pulmonary AVMs (2-6). It is thought that the cardiac failure associated with hepatic AVMs is reversible (14) so it has been recommended that these patients be delivered as soon as feasible (7). Cardiac failure can be treated with diuretics, however close consultation is required between the cardiology and obstetric teams because reduced uterine artery perfusion and foetal oxygen delivery are potential consequences. In our patient, normal cardiac function did not return after the birth of her first child.

In deciding on the method of anaesthesia for caesarean section or analgesia for labour, the risks of the various techniques must be considered in light of the patient's condition, in particular the presence of spinal AVMs or acute cardiac decompensation, with few publications about the anaesthetic management of parturients with high output cardiac failure secondary to AVMs. The goals are to maintain cardiac contractility and avoid sudden changes in afterload and preload. Central neuraxial block has the advantage of reducing afterload and preload but is not well described in these patients. The incidence of spinal AVMs is up to 1 to 2% (1), and these should be excluded by magnetic resonance imaging before neuraxial anaesthesia is performed (3,6). In this patient spinal magnetic resonance imaging had never been performed. In the first pregnancy, a neuraxial technique was not chosen for the reasons discussed shortly. In preparation for the second pregnancy, multidisciplinary consensus was that the stability of general anaesthesia associated with the first delivery justified general anaesthesia again.

The emergent nature of the patient's first presentation, with worsening cardiac failure, necessitated semi-urgent delivery via caesarean section. A general anaesthetic technique was chosen because the patient could not lie flat due to severe orthopnoea. Furthermore, neuraxial anaesthesia has not been well described in this subset of patients and the effects were unpredictable. An opioid-based anaesthetic facilitated cardiovascular stability (15) during the intubation and surgery and good communication with the neonatologist occurred. ICU admission was planned for mechanical ventilation and haemodynamic stabilisation prior to likely improvement of cardiac function over the course of several postpartum days. Remifentanil (16) and alfentanil (17) have been successfully used in general anaesthesia techniques in parturients with heart disease undergoing caesarean section. Fentanyl was chosen by the cardiac anaesthetists in our patient because of their familiarity with it and their opinion that it provides the best haemodynamic stability. A PAC was inserted to monitor cardiac function and to guide fluid and inotrope therapy. PACs can be useful in parturients with cardiac disease (18,19) because central venous pressure is a poor indicator of left ventricular end-diastolic pressure. Although there are risks from these catheters, these are low in our centre where the medical and nursing staff are proficient in managing the catheters and interpreting the data (18,19). TOE has been used in a variety of non-cardiac surgeries (20) and is a category II indication in the presence of an increased risk of haemodynamic disturbance (21). Morbidity associated with intraoperative TOE is low (22) and was acceptable in this patient who had no history of oesophageal disease or upper gastrointestinal AVMs. We consider the combination of PAC and TOE provided information on haemodynamic pressures and cardiac structure that enhanced management.

Multidisciplinary team management, close monitoring and planned caesarean birth under general anaesthesia at 30 weeks gestation in the second pregnancy optimised conditions. The early involvement and vigilance of various health professionals during her second pregnancy may have led to a better outcome compared with her first pregnancy, as evidenced by her earlier discharge from the ICU and hospital. Due to the complex nature of patients with HHT, these patients should be managed in a tertiary environment.

Accepted for publication on August 28, 2009.


(1.) Begbie ME, Wallace GMF, Shovlin CL. Hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J 2003; 79:18-24.

(2.) Jakobi P, Weiner Z, Best L, Itskovitz-Eldor J. Hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations. Obstet Gynecol 2001; 97:813-814.

(3.) Shovlin CL, Sodhi V, McCarthy A, Lasjaunias P, Jackson JE, Sheppard MN. Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (OslerWeber-Rendu syndrome): suggested approach for obstetric services. BJOG 2008; 115:1108-1115.

(4.) Shovlin CL, Winstock AR, Peters AM, Jackson JE, Hughes JM. Medical complications of pregnancy in hereditary haemorrhagic telangiectasia. QJM 1995; 88:879-87.

(5.) Worda C, Lang I, Husslein P, Kneussl M. Hereditary hemorrhagic telangiectasia and pregnancy. Obstet Gynecol 2007; 110:474-477.

(6.) El Shobary H, Schricker T, Kaufman I. Anaesthetic management of parturients with hereditary haemorrhagic telangiectasia for caesarean section. Int J Obstet Anesth 2009; 18:176-181.

(7.) Garcia-Tsao G. Liver involvement in hereditary hemorrhagic telangiectasia (HHT). J Hepatol 2007; 46:499-507.

(8.) Triulzi MO, Gillam LD, Gentile R. Normal adult cross-sectional echocardiographic values: linear dimensions and chamber areas. Echocardiography 1984; 1:403-426.

(9.) Guise JM, Segel S. Teamwork in obstetric critical care. Best Pract Res Clin Obstet Gynaecol 2008; 22:937-951.

(10.) Buscarini E, Danesino C, Olivieri C, Lupinacci G, Zambelli A. Liver involvement in hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber disease. Dig Liver Dis 2005; 37:635-645.

(11.) Miller FJ Jr, Whiting JH, Korzenik JR, White RI. Caution with use of hepatic embolization in the treatment of hereditary hemorrhagic telangiectasia. Radiology 1999; 213:928-930.

(12.) Thevenot T, Vanlemmens C, Di Martino V, Becker M, Denue P, Kantelip B et al. Liver transplantation for cardiac failure in patients with hereditary hemorrhagic telangiectasia. Liver Transpl 2005; 11:834-838.

(13.) Hillert C, Broering DC, Gundlach M, Knoefel WT, Izbicki JR, Rogiers X. Hepatic involvement in hereditary hemorrhagic telangiectasia: an unusual indication for liver transplantation. Liver Transpl 2001; 7:266-268.

(14.) Livneh A, Langevitz P, Morag B, Catania A, Pras M. Functionally reversible hepatic arteriovenous fistulas during pregnancy in patients with hereditary hemorrhagic telangiectasia. South Med J 1988; 81:1047-1049.

(15.) Mushlin PS, Davidson KM. Cardiovascular disease in pregnancy. In: Datta S, ed. Anesthetic and Obstetric Management of High-risk Pregnancy. 3rd ed. Springer-Verlag New York Inc. 2004. p. 164-165.

(16.) Wadsworth R, Greer R, MacDonald JMS, Vohra A. The use of remifentanil during general anaesthesia for caesarean delivery in two patients with severe heart dysfunction. Int J Obstet Anesth 2002; 11:38-43.

(17.) Esmail MM, Catling S, Mahon S, Dingley J. Anaesthetic management of caesarean section in a patient with a permanent pacemaker and severe bilateral ventricular dilatation. Int J Obstet Anesth 2000; 9:51-54.

(18.) Clark SL, Horenstein JM, Phelan JP, Montag TW, Paul RH. Experience with the pulmonary artery catheter in obstetrics and gynecology. Am J Obstet Gynecol 1985; 152:374-378.

(19.) Practice guidelines for pulmonary artery catheterization. A report by the American Society of Anesthesiologists Task Force on Pulmonary Artery Catheterization. Anesthesiology 1993; 78:380-394.

(20.) Mahmood F, Christie A, Matyal R. Transesophageal echocardiography and noncardiac surgery. Semin Cardiothorac Vasc Anesth 2008; 12:265-289.

(21.) Practice guidelines for perioperative transesophageal echocardiography. A report by the American Society of Anesthesiologists and the Society of Cardiovascular Anesthesiologists Task Force on Transesophageal Echocardiography. Anesthesiology 1996; 84:986-1006.

(22.) Kallmeyer IJ, Collard CD, Fox JA, Body SC, Shernan SK. The safety of intraoperative transesophageal echocardiography: a case series of 7200 cardiac surgical patients. Anesth Analg 2001; 92:1126-1130.

C. F. LAI *, A. DENNIS [[dagger]], J. GRAHAM [[double dagger]] Department of Anaesthesia, Austin Health, Heidelberg, Victoria, Australia

* M.B., B.S., Provisional Fellow.

[[dagger]] M.B., B.S., P.G.Dip.Echo., F.A.N.Z.C.A., Deputy Head of Anaesthesia, Mercy Hospital for Women.

[[double dagger]] M.B., B.S., F.A.N.Z.C.A., Consultant Anaesthetist.

Address for correspondence: Dr C. Lai, email:
COPYRIGHT 2010 Australian Society of Anaesthetists
No portion of this article can be reproduced without the express written permission from the copyright holder.
Copyright 2010 Gale, Cengage Learning. All rights reserved.

Article Details
Printer friendly Cite/link Email Feedback
Author:Lai, C.F.; Dennis, A.; Graham, J.
Publication:Anaesthesia and Intensive Care
Article Type:Case study
Date:Mar 1, 2010
Previous Article:Cerebral flow pattern monitoring by transcranial Doppler during cardiopulmonary resuscitation.
Next Article:Plasmapheresis treatment in Guillain-Barre syndrome: potential benefit over intravenous immunoglobulin.

Terms of use | Privacy policy | Copyright © 2018 Farlex, Inc. | Feedback | For webmasters