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Management of ALCAPA in two pregnancies
International Journal of Cardiology 181 (2015) 353–354
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Management of ALCAPA in two pregnancies☆ Matthew Cauldwell a,⁎, Lorna Swan b, Kate von Klemperer c, Roshni Patel d, Phil Steer a a
Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, London, United Kingdom Clinical Lead for Adult Congenital Heart Disease, Royal Brompton Hospital, London SW3 6NP, United Kingdom c Royal Brompton Hospital, London, United Kingdom d Division of Women's and Children, Chelsea and Westminster Hospital, 369 Fulham Road, London SW10 9NH, United Kingdom b
a r t i c l e
i n f o
Article history: Received 17 December 2014 Accepted 21 December 2014 Available online 24 December 2014 Keywords: Pregnancy ALCAPA Management
12 weeks and then again at 20 weeks and showed no change. A plan was made for a trial of vaginal delivery with a recommendation for an epidural and but to limit pushing in the second stage. She went into spontaneous labour at 39 weeks of gestation. She progressed to full dilatation and after 60 min of pushing delivered a female infant (3460 g) with Apgar scores of 9 (1 min) and 10 (5 min). Following delivery she was transferred to the Obstetric High Dependency Unit (HDU) and was observed for a period of 24 h. She remained an inpatient for two further days. Pre-discharge echo showed no change in her LV function. 2. Case 2
1. Case 1 A nulliparous lady booked under the joint obstetric cardiac (JOC) team at 7 weeks of gestation. The diagnosis of ALCAPA syndrome was made when she presented with infantile myocarditis. She had a Takeuchi repair (intrapulmonary aortocoronary tunnel) procedure at the age of 6 weeks. She had further surgery at the age of 8 years to repair pulmonary stenosis and a coronary artery fistula. At the age of 17 she represented with angina and required a LIMA to LAD bypass graft. Prior to pregnancy the patient was asymptomatic apart from shortlived palpitations. She exercised regularly with no limitation. She had received preconception counselling from her cardiac obstetric team and was expected to do well. Her echo and CMR prior to pregnancy showed maintained left ventricular function systolic with an anteroapical region of hypokinesis and prior to conception her perindopril therapy was discontinued. There was mild to moderate supravalvar pulmonary stenosis with a peak gradient of 49 mm Hg. She was reviewed in the joint clinic initially at 4 weekly intervals and then every week from 32 weeks. Echocardiography was performed at
☆ Author note: Consent was obtained from both individuals prior to submission. ⁎ Corresponding author at: Academic Department of Obstetrics and Gynaecology, 3rd Floor, Chelsea and Westminster Hospital, 369 Fulham Road, London SW10 9NH, United Kingdom. E-mail address: [email protected] (M. Cauldwell).
http://dx.doi.org/10.1016/j.ijcard.2014.12.081 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
A nulliparous lady booked under the JOC team at 11 weeks of gestation. She had a Takeuchi repair performed aged 20 years of age following a diagnosis of ALCAPA which was made after a failed army medical. She was under yearly review by the cardiologists. Maternal echo was performed in the first trimester and showed good left ventricular. She was reviewed regularly and remained symptom free. Foetal growth scans demonstrated normal growth in the third trimester. A decision was made for a trial of vaginal delivery with limited pushing. She went into spontaneous labour at 40 weeks of gestation. She had an epidural in early labour. She progressed rapidly in the first stage of labour to full dilatation and after 7 min of pushing delivered a live male infant (3500 g) with Apgar scores of 9 (1 min) and 10 (5 min). She was admitted to the Obstetric HDU for a 24 hour period and was discharged the following day. ALCAPA is a rare occurring in around 1 in 300,000 cases of congenital heart disease [1]. It commonly requires surgical repair involving the relocation or ligation of the abnormal vessel and the establishment of alternative coronary perfusion if needed. Fig. 1 shows a cardiac CT angiogram of an unrepaired ALCAPA. Reported cases of pregnancies in mothers with the condition are few. Clinical management by an experienced multidisciplinary team is required [2,3]. No long-term studies of large populations of adults with corrected ALCAPA are available, but the prognosis is generally good. Patients should undergo preconception counselling. However given the rarity of this condition, specific guidance regarding management cannot be evidence based. The crucial issues determining outcome are the extent of LV dysfunction (from previous myocardial damage) and the degree of reversible ischaemia. Careful management within a team of cardiologists,
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M. Cauldwell et al. / International Journal of Cardiology 181 (2015) 353–354
demand associated with repeat Valsalva manoeuvre in those with impaired cardiac reserve [4]. As there is a risk of postpartum arrhythmia, particularly in those with left ventricular dysfunction, patients should be monitored postnatally in a HDU setting. There are also needs to be a clear process for following up these patients after delivery and we would suggest early follow-up postnatally at around 6–12 weeks after delivery. The risk of recurrence of congenital heart disease in the foetus should also not be forgotten. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References
Fig. 1. CT Angiogram of an unrepaired ALCAPA.
obstetricians and anaesthetists who are familiar with caring for women with congenital heart disease is key. Obstetric management should include consideration for shortening the second stage to reduce cardiac
[1] J. Pfannschmidt, H. Ruskowski, E.R. de Vivie, Bland–White–Garland syndrome. Clinical aspects, diagnosis, therapy, Klin. Padiatr. 204 (1992) 328–334 (German). [2] C.K. Choong, C. Martinez, H.B. Barner, P.A. Ludbrook, Bland–White–Garland syndrome in pregnancy: reoperation of ALCAPA with an internal thoracic radial artery “Y”-graft, Ann. Thorac. Surg. 81 (4) (Apr 2006) 1512–1514. [3] V.R. Klein, J.T. Repke, G.P. Marquette, J.R. Niebyl, The Bland–White–Garland syndrome in pregnancy, Am. J. Obstet. Gynecol. 150 (1) (Sep 1 1984) 106–107. [4] P, P.G. Pieper, P. Presbitero, J.W. Roos-Hesselink, M. Schaufelberger, U. Seeland, L. Torracca, ESC Committee for Practice Guidelines, ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC), Eur. Heart J. 32 (24) (Dec 2011) 3147–3197.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Management of ALCAPA in two pregnancies☆ Matthew Cauldwell a,⁎, Lorna Swan b, Kate von Klemperer c, Roshni Patel d, Phil Steer a a
Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, London, United Kingdom Clinical Lead for Adult Congenital Heart Disease, Royal Brompton Hospital, London SW3 6NP, United Kingdom c Royal Brompton Hospital, London, United Kingdom d Division of Women's and Children, Chelsea and Westminster Hospital, 369 Fulham Road, London SW10 9NH, United Kingdom b
a r t i c l e
i n f o
Article history: Received 17 December 2014 Accepted 21 December 2014 Available online 24 December 2014 Keywords: Pregnancy ALCAPA Management
12 weeks and then again at 20 weeks and showed no change. A plan was made for a trial of vaginal delivery with a recommendation for an epidural and but to limit pushing in the second stage. She went into spontaneous labour at 39 weeks of gestation. She progressed to full dilatation and after 60 min of pushing delivered a female infant (3460 g) with Apgar scores of 9 (1 min) and 10 (5 min). Following delivery she was transferred to the Obstetric High Dependency Unit (HDU) and was observed for a period of 24 h. She remained an inpatient for two further days. Pre-discharge echo showed no change in her LV function. 2. Case 2
1. Case 1 A nulliparous lady booked under the joint obstetric cardiac (JOC) team at 7 weeks of gestation. The diagnosis of ALCAPA syndrome was made when she presented with infantile myocarditis. She had a Takeuchi repair (intrapulmonary aortocoronary tunnel) procedure at the age of 6 weeks. She had further surgery at the age of 8 years to repair pulmonary stenosis and a coronary artery fistula. At the age of 17 she represented with angina and required a LIMA to LAD bypass graft. Prior to pregnancy the patient was asymptomatic apart from shortlived palpitations. She exercised regularly with no limitation. She had received preconception counselling from her cardiac obstetric team and was expected to do well. Her echo and CMR prior to pregnancy showed maintained left ventricular function systolic with an anteroapical region of hypokinesis and prior to conception her perindopril therapy was discontinued. There was mild to moderate supravalvar pulmonary stenosis with a peak gradient of 49 mm Hg. She was reviewed in the joint clinic initially at 4 weekly intervals and then every week from 32 weeks. Echocardiography was performed at
☆ Author note: Consent was obtained from both individuals prior to submission. ⁎ Corresponding author at: Academic Department of Obstetrics and Gynaecology, 3rd Floor, Chelsea and Westminster Hospital, 369 Fulham Road, London SW10 9NH, United Kingdom. E-mail address: [email protected] (M. Cauldwell).
http://dx.doi.org/10.1016/j.ijcard.2014.12.081 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved.
A nulliparous lady booked under the JOC team at 11 weeks of gestation. She had a Takeuchi repair performed aged 20 years of age following a diagnosis of ALCAPA which was made after a failed army medical. She was under yearly review by the cardiologists. Maternal echo was performed in the first trimester and showed good left ventricular. She was reviewed regularly and remained symptom free. Foetal growth scans demonstrated normal growth in the third trimester. A decision was made for a trial of vaginal delivery with limited pushing. She went into spontaneous labour at 40 weeks of gestation. She had an epidural in early labour. She progressed rapidly in the first stage of labour to full dilatation and after 7 min of pushing delivered a live male infant (3500 g) with Apgar scores of 9 (1 min) and 10 (5 min). She was admitted to the Obstetric HDU for a 24 hour period and was discharged the following day. ALCAPA is a rare occurring in around 1 in 300,000 cases of congenital heart disease [1]. It commonly requires surgical repair involving the relocation or ligation of the abnormal vessel and the establishment of alternative coronary perfusion if needed. Fig. 1 shows a cardiac CT angiogram of an unrepaired ALCAPA. Reported cases of pregnancies in mothers with the condition are few. Clinical management by an experienced multidisciplinary team is required [2,3]. No long-term studies of large populations of adults with corrected ALCAPA are available, but the prognosis is generally good. Patients should undergo preconception counselling. However given the rarity of this condition, specific guidance regarding management cannot be evidence based. The crucial issues determining outcome are the extent of LV dysfunction (from previous myocardial damage) and the degree of reversible ischaemia. Careful management within a team of cardiologists,
354
M. Cauldwell et al. / International Journal of Cardiology 181 (2015) 353–354
demand associated with repeat Valsalva manoeuvre in those with impaired cardiac reserve [4]. As there is a risk of postpartum arrhythmia, particularly in those with left ventricular dysfunction, patients should be monitored postnatally in a HDU setting. There are also needs to be a clear process for following up these patients after delivery and we would suggest early follow-up postnatally at around 6–12 weeks after delivery. The risk of recurrence of congenital heart disease in the foetus should also not be forgotten. Conflict of interest The authors report no relationships that could be construed as a conflict of interest. References
Fig. 1. CT Angiogram of an unrepaired ALCAPA.
obstetricians and anaesthetists who are familiar with caring for women with congenital heart disease is key. Obstetric management should include consideration for shortening the second stage to reduce cardiac
[1] J. Pfannschmidt, H. Ruskowski, E.R. de Vivie, Bland–White–Garland syndrome. Clinical aspects, diagnosis, therapy, Klin. Padiatr. 204 (1992) 328–334 (German). [2] C.K. Choong, C. Martinez, H.B. Barner, P.A. Ludbrook, Bland–White–Garland syndrome in pregnancy: reoperation of ALCAPA with an internal thoracic radial artery “Y”-graft, Ann. Thorac. Surg. 81 (4) (Apr 2006) 1512–1514. [3] V.R. Klein, J.T. Repke, G.P. Marquette, J.R. Niebyl, The Bland–White–Garland syndrome in pregnancy, Am. J. Obstet. Gynecol. 150 (1) (Sep 1 1984) 106–107. [4] P, P.G. Pieper, P. Presbitero, J.W. Roos-Hesselink, M. Schaufelberger, U. Seeland, L. Torracca, ESC Committee for Practice Guidelines, ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC), Eur. Heart J. 32 (24) (Dec 2011) 3147–3197.