Pregnancy and Pulmonary Hypertension

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Anesthesiology. 2005 Jun;102(6):1133-7; discussion 5A-6A.
Comment in: Anesthesiology. 2006 Feb;104(2):382; author reply 383.
Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases.
Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jais X, Humbert M, Audibert F, Frydman R, Simonneau G, Benhamou D.
Department of Anesthesiology and Intensive Care, Antoine Beclere Hospital-Assistance Publique des Hopitaux de Paris (APHP), 157 rue de la Porte de Trivaux, 92141 Clamart Cedex, BP 405, France.

BACKGROUND: Available literature on pregnant women with severe pulmonary hypertension (PH) relies mainly on anecdotal case reports and two series only. METHODS: The authors reviewed the charts of all pregnant women with severe PH who were followed up at their institution during the past 10 yr, to assess the multidisciplinary treatment and outcome of these patients. RESULTS: Fifteen pregnancies in 14 women with severe PH were managed during this period: There were 4 cases of idiopathic pulmonary arterial hypertension (PAH), 6 cases of congenital heart disease-associated PAH, 1 case of fenfluramine-associated PAH, 1 case of mixed connective tissue-associated PAH, 1 case of human immunodeficiency virus-associated PAH, and 2 cases of chronic thromboembolic PH. PH presented during pregnancy in 3 patients. Two patients died before delivery at 12 and 23 weeks' gestation. Four patients had vaginal deliveries with regional anesthesia: One died 3 months postpartum, one worsened, and two remained stable. Four had cesarean deliveries during general anesthesia: One died 3 weeks postpartum, one worsened, and two remained stable. Five had cesarean deliveries during low-dose combined spinal-epidural anesthesia: One died 1 week postpartum, and four remained stable. There were two fetal deaths: one related to therapeutic abortion at 21 weeks' gestation and one stillbirth at 36 weeks' gestation followed by the death of the mother 1 week later. CONCLUSIONS: Despite the most modern treatment efforts, the maternal mortality was 36%. Scheduled cesarean delivery during combined spinal-epidural anesthesia seemed to be an attractive approach, but there was no evidence of actual benefit. Therefore, pregnancy must still be discouraged in patients with severe PH.

Bol Asoc Med P R. 2005 Oct-Dec;97(4):328-33.
Primary pulmonary hypertension and pregnancy.
Carro-Jimenez EJ, Lopez JE.
Department of Medicine, Cardiology Section, University of Puerto Rico School of Medicine, San Juan, Puerto Rico.

Primary pulmonary hypertension (PPH) is a disorder intrinsic to the pulmonary vascular bed characterized by sustained elevation in pulmonary artery pressure and pulmonary vascular resistance with normal pulmonary artery wedge pressure, in the absence of a known cause. Cardiovascular disorders are the most common cause of morbidity and mortality in pregnant women. Risk is so great with some cardiovascular abnor-malities that recommendation of avoidance or interruption of pregnancy is supportable, one of these conditions is pulmonary hypertension. In this article two patients with primary pulmonary hypertension who sustained two pregnancies each are described. Both patients had uneventful pregnancies and deliveries, with all neonates surviving. When evaluating primary pulmonary hypertension and pregnancy, early studies reported a maternal mortality rate as high as 50%. More recent studies report a maternal mortality of 30%. In this article, cases of primary pulmonary hypertension undergoing pregnancy from 1978 to 2005 were reviewed, revealing a 22% maternal mortality for the total number of pregnancies. Despite advances in treatment, primary pulmonary hypertension continues to carry considerable maternal morbidity and mortality, and prevention of pregnancy is still the main recommendation. Early diagnosis and treatment is critical and a multi-disciplinary approach is required when dealing with a patient with PPH who desires to continue pregnancy.

J Heart Valve Dis. 2001 May;10(3):288-93.
Role of closed mitral commissurotomy in mitral stenosis with severe pulmonary hypertension.
Sajja LR, Mannam GC.
Division of Cardiothoracic Surgery, City Cardiac Research Centre, Vijayawada, Andhra Pradesh, India.

BACKGROUND AND AIMS OF THE STUDY: Closed mitral commissurotomy (CMC) is a well-established method for treatment of rheumatic mitral stenosis, but outcome in patients with severe pulmonary arterial hypertension (PAH) has not been clearly documented. METHODS: Between April 1996 and October 1999, among 61 patients who underwent CMC, 27 had severe PAH (systolic pressure > 100 mmHg). Of these patients, 11 were in NYHA class III, and 16 were in class IV. Preoperatively, the mean pulmonary artery (PA) pressure was 107.85 +/- 5.74 mmHg (range: 100-118 mmHg), mitral valve area (MVA) 0.704 +/- 0.106 cm2 (range: 0.5-0.91 cm2), and transmitral gradient 11.93 +/- 1.54 mmHg (range: 10-15 mmHg). The echocardiographic mitral valve score was 6.37 +/- 1.11 (range: 6-10). RESULTS: There was no operative mortality or incidence of significant (> or = 2+) post-CMC mitral regurgitation or cerebrovascular accident. The MVA increased to 2.385 +/- 0.248 cm2 (range: 1.9-2.8 cm2), the transmitral gradient fell to 2.44 +/- 0.51 mmHg (range: 2-3 mmHg), and postoperative PA systolic pressure fell to 33.33 +/- 8.20 mmHg (range: 30-60 mmHg). During a mean follow up of 26.9 months (range: 11-51 months), 23 patients were in NYHA class I and four were in class II. There were no significant differences in parameters between sexes, but mean male age was five years less than mean female age. CONCLUSION: In the subset of patients with severe PAH, surgical CMC is a safe and effective procedure that results in greater MVA and a more significant and sustained fall in PA pressure compared with reported series of percutaneous balloon mitral valvuloplasty.

Acta Anaesthesiol Belg. 2001;52(2):207-9.
Target controlled infusion of remifentanil and propofol for cesarean section in a patient with multivalvular disease and severe pulmonary hypertension.
Mertens E, Saldien V, Coppejans H, Bettens K, Vercauteren M.
Dept. of Anesthesia, University Hospital Antwerp, Wilrijkstraat 10, B-2650 Edegem, Belgium.

A 36 year old parturient with known valvular heart disease was admitted with respiratory distress and fatigue after 35 weeks of pregnancy. Echocardiography revealed severe tricuspid regurgitation, mitral stenosis and aortic valve insufficiency. Following clinical examination and insertion of a radial and pulmonary artery catheter it was decided to perform a Caesarean Section. The pulmonary artery pressure upon arrival in the operating theatre was 105/50 mm Hg whereas cardiac output was 3.5 l/min. Induction of anesthesia was performed with a target controlled infusion of remifentanil and propofol combined with rocuronium bromide. Haemodynamic variables remained very stable during and after intubation. The lungs of the apnoeic baby were manually ventilated until spontaneous respiration began at 1 minute post delivery. Apgar scores were 3, 7 and 9 after 1, 5 and 10 minutes respectively. Umbilical artery pH was 7.29. The patient's haemodynamic status gradually improved over the following few days. Two months following delivery she underwent unevenful valvular surgery.

Am J Obstet Gynecol. 1998 Jul;179(1):270-2.
Emergency mitral valve replacement in the setting of severe pulmonary hypertension and acute cardiovascular decompensation after evacuation of twins at fifteen weeks' gestation.
Tio I, Tewari K, Balderston KD, Milliken JC, Porto M.
Department of Obstetrics and Gynecology, University of California, Irvine, Medical Center, Orange 92868, USA.

A case of critical mitral stenosis with severe pulmonary hypertension in a twin pregnancy is presented. On therapeutic evacuation at 15 weeks' gestation the patient became critically unstable, necessitating emergency open heart surgery with mitral valve replacement.

Can J Anaesth. 1990 Sep;37(6):685-8.
Alfentanil for urgent caesarean section in a patient with severe mitral stenosis and pulmonary hypertension.
Batson MA, Longmire S, Csontos E.
Department of Anesthesiology, Baylor College of Medicine, Houston, Texas 77030.

We present the case of a parturient with severe mitral stenosis and pulmonary hypertension who received general anaesthesia using alfentanil for urgent Caesarean section. Alfentanil promoted haemodynamic stability and allowed immediate postoperative extubation. Epidural morphine provided postoperative analgesia. This combination permitted early ambulation and prevention of thromboembolism. A disadvantage of this technique, neonatal respiratory depression, was promptly reversed with a single dose of naloxone. The anaesthetic management of mitral stenosis in pregnancy is discussed and the neonatal pharmacokinetics of maternally administered alfentanil are presented.

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