Right heart failure in pregnant women with cyanotic congenital heart disease — The good, the bad and the ugly E. Zengin, C. Sinning, B. Schrage, G.C. Mueller, H. Klose, J. Sachweh, M. Goepfert, B. Hueneke, S. Blankenberg, R. Kozlik-Feldmann PII: DOI: Reference:
S0167-5273(15)30601-X doi: 10.1016/j.ijcard.2015.10.036 IJCA 21326
To appear in:
International Journal of Cardiology
Received date: Accepted date:
19 September 2015 4 October 2015
Please cite this article as: Zengin E, Sinning C, Schrage B, Mueller GC, Klose H, Sachweh J, Goepfert M, Hueneke B, Blankenberg S, Kozlik-Feldmann R, Right heart failure in pregnant women with cyanotic congenital heart disease — The good, the bad and the ugly, International Journal of Cardiology (2015), doi: 10.1016/j.ijcard.2015.10.036
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
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The Good, the Bad and the Ugly
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Right heart failure in pregnant women with cyanotic congenital heart disease –
Zengin1♠∞ E, Sinning1♠ C, Schrage1 B, Mueller G2 C, Klose H3, Sachweh4 J, Goepfert5 M, Hueneke6
NU
B, Blankenberg1 S, and Kozlik-Feldmann2 R 1
Department of General and Interventional Cardiology Department of Pediatric Cardiology 3 Department of Internal Medicine 2, Section of Pneumology 4 Department of Cardiovascular Surgery 5 Department of Anesthesiology 6 Department of Obstetrics and Fetal Medicine
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♠ Contributed equally
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D
MA
2
∞Corresponding author:
AC
Elvin Zengin, MD University Heart Center Hamburg Department of General and Interventional Cardiology Martinistr. 52 20246 Hamburg Tel:004915222816423 Mail:[email protected]
ACCEPTED MANUSCRIPT Management of pregnant patients with cyanotic congenital heart disease is still a challenge as pulmonary-arterial hypertension (PAH) limits the outcome for the mother and the child [1, 2]. In this disease spectrum tetralogy of fallot (TOF) is the most common besides Eisenmenger reaction resulting
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from shunt lesions like ventricular septum defect or malformations of the aorta with the truncus arteriosus [3, 4]. Remodeling of the right ventricle is a condition often encountered during and after
SC R
pregnancy and thus has to be reconciled in the management of congenital heart disease [3, 5]. We summarize three cases of women being referred during pregnancy with cyanotic congenital heart
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disease and PAH.
The first case is of a 28-year old woman with a non-restrictive ventricle septum defect surgically
MA
corrected at the age of 5 years and known PAH Nice Class 1. The patient was medicated with a combination of endothelin receptor antagonist (ERA) and phosphodiasterase-5 inhibitor (PPI5).
D
Gradient derived from tricuspid regurgitation was stable with 90mmHg. Upon reporting to be
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pregnant, ERA was stopped and medication was continued on PPI5. During pregnancy systolic pulmonary-arterial pressure (sPAP) derived from echocardiography remained 90mmHg and the patient
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was hospitalized for further control of ongoing pregnancy. The patient did give birth in the 34 th week of pregnancy with Caesarean section and consented to sterilization in the same operation. After 5
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month with combination therapy with ERA and PPI5 she shows a stable hemodynamic situation with a measured sPAP of 60mmHg. The second case reports the medical history of a 32-year old woman with truncus arteriosus type 1 which was not corrected in the German Democratic Republic due to the believed early fatal outcome. The patient had Eisenmenger reaction and PAH Nice Class 1 upon presentation but refused specific PAH medication. The patient presented due to planned pregnancy to our outpatient clinic. As consequence of the high-risk pregnancy the patient was hospitalized in the 27th week of pregnancy. Due to the Eisenmenger physiology sPAP was measured with 100mmHg in echocardiography. As reason of the severe placenta insufficiency Caesarean section was planned and performed in the 30 th week of pregnancy in the cardiovascular operation theatre. After 1,5 years the patient had no further
ACCEPTED MANUSCRIPT impairment of her everyday activities but still refuses specific PAH medication with a measured sPAP of still 90-100mmHg.
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The third case describes the pregnancy of a 32-year old patient with TOF palliated during the first year
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of live with surgical atrioseptostomy and a Waterston shunt until surgical correction at the age of 7. At
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the age of 25 the patient showed a mean pulmonary arterial pressure of 22mmHg and thus borderline PAH in right heart catheter. In the outpatient clinic no contraindication against pregnancy was reported although the patient had severe regurgitation of the pulmonic valve but sPAP was not elevated in
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echocardiography. During pregnancy the patient was increasingly limited in physical activities and did gain weight with ankle and leg swelling. The patient had to stay in hospital after completion of the 26th
MA
week of pregnancy and had a maximum sPAP of 80-90mmHg derived from echocardiography. As a consequence diuretics were administered and due to placental insufficiency, Caesarean section was
D
performed in the 32th week of pregnancy because of constant worsening of the fetus. Afterwards, the
TE
patient had a prolonged intensive care unit stay with combination therapy of ERA and PPI5 and still elevated sPAP with 60mmHg. Right and left heart catheterization after birth did show near to systemic
CE P
pressure in the pulmonary arteries, elevated left ventricular filling pressures with 35mmHg and a central venous pressure of 30mmHg. Following a long hospital stay the patient is on PAH combination
AC
therapy and is listed for heart and lung transplantation. (Figure 1 shows the echocardiography in each case before and after pregnancy). Pulmonary-arterial hypertension has a plethora of facets which have to be accounted for in congenital and especially cyanotic heart disease [1, 3]. The cases illustrate the difficult circumstances which have to be overcome by the patients and also the treating physicians. Patients which were advised not to become pregnant did decide to do so and were treated as high-risk pregnancy in a multidisciplinary team and delivered successfully. However, on the contrary one patient with a supposed low-risk pregnancy in cyanotic congenital heart disease had an extreme adverse course, illustrating the potential risk of remodeling of the right ventricle during pregnancy [5].
Conflicts of Interest: None
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References
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[1] Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart JM, et al. 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. 2011;32:3147-97. [2] Schmaltz AA, Neudorf U, Winkler UH. Outcome of pregnancy in women with congenital heart disease. Cardiol Young. 1999;9:88-96. [3] Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31:2915-57. [4] Niwa K, Perloff JK, Kaplan S, Child JS, Miner PD. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol. 1999;34:223-32. [5] Egidy Assenza G, Cassater D, Landzberg M, Geva T, Schreier J, Graham D, et al. The effects of pregnancy on right ventricular remodeling in women with repaired tetralogy of Fallot. Int J Cardiol. 2013;168:1847-52.
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Figure 1 Echocardiography in case 1(A),2(B) and 3(C)
S0167-5273(15)30601-X doi: 10.1016/j.ijcard.2015.10.036 IJCA 21326
To appear in:
International Journal of Cardiology
Received date: Accepted date:
19 September 2015 4 October 2015
Please cite this article as: Zengin E, Sinning C, Schrage B, Mueller GC, Klose H, Sachweh J, Goepfert M, Hueneke B, Blankenberg S, Kozlik-Feldmann R, Right heart failure in pregnant women with cyanotic congenital heart disease — The good, the bad and the ugly, International Journal of Cardiology (2015), doi: 10.1016/j.ijcard.2015.10.036
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
SC R
IP
The Good, the Bad and the Ugly
T
Right heart failure in pregnant women with cyanotic congenital heart disease –
Zengin1♠∞ E, Sinning1♠ C, Schrage1 B, Mueller G2 C, Klose H3, Sachweh4 J, Goepfert5 M, Hueneke6
NU
B, Blankenberg1 S, and Kozlik-Feldmann2 R 1
Department of General and Interventional Cardiology Department of Pediatric Cardiology 3 Department of Internal Medicine 2, Section of Pneumology 4 Department of Cardiovascular Surgery 5 Department of Anesthesiology 6 Department of Obstetrics and Fetal Medicine
CE P
♠ Contributed equally
TE
D
MA
2
∞Corresponding author:
AC
Elvin Zengin, MD University Heart Center Hamburg Department of General and Interventional Cardiology Martinistr. 52 20246 Hamburg Tel:004915222816423 Mail:[email protected]
ACCEPTED MANUSCRIPT Management of pregnant patients with cyanotic congenital heart disease is still a challenge as pulmonary-arterial hypertension (PAH) limits the outcome for the mother and the child [1, 2]. In this disease spectrum tetralogy of fallot (TOF) is the most common besides Eisenmenger reaction resulting
IP
T
from shunt lesions like ventricular septum defect or malformations of the aorta with the truncus arteriosus [3, 4]. Remodeling of the right ventricle is a condition often encountered during and after
SC R
pregnancy and thus has to be reconciled in the management of congenital heart disease [3, 5]. We summarize three cases of women being referred during pregnancy with cyanotic congenital heart
NU
disease and PAH.
The first case is of a 28-year old woman with a non-restrictive ventricle septum defect surgically
MA
corrected at the age of 5 years and known PAH Nice Class 1. The patient was medicated with a combination of endothelin receptor antagonist (ERA) and phosphodiasterase-5 inhibitor (PPI5).
D
Gradient derived from tricuspid regurgitation was stable with 90mmHg. Upon reporting to be
TE
pregnant, ERA was stopped and medication was continued on PPI5. During pregnancy systolic pulmonary-arterial pressure (sPAP) derived from echocardiography remained 90mmHg and the patient
CE P
was hospitalized for further control of ongoing pregnancy. The patient did give birth in the 34 th week of pregnancy with Caesarean section and consented to sterilization in the same operation. After 5
AC
month with combination therapy with ERA and PPI5 she shows a stable hemodynamic situation with a measured sPAP of 60mmHg. The second case reports the medical history of a 32-year old woman with truncus arteriosus type 1 which was not corrected in the German Democratic Republic due to the believed early fatal outcome. The patient had Eisenmenger reaction and PAH Nice Class 1 upon presentation but refused specific PAH medication. The patient presented due to planned pregnancy to our outpatient clinic. As consequence of the high-risk pregnancy the patient was hospitalized in the 27th week of pregnancy. Due to the Eisenmenger physiology sPAP was measured with 100mmHg in echocardiography. As reason of the severe placenta insufficiency Caesarean section was planned and performed in the 30 th week of pregnancy in the cardiovascular operation theatre. After 1,5 years the patient had no further
ACCEPTED MANUSCRIPT impairment of her everyday activities but still refuses specific PAH medication with a measured sPAP of still 90-100mmHg.
T
The third case describes the pregnancy of a 32-year old patient with TOF palliated during the first year
IP
of live with surgical atrioseptostomy and a Waterston shunt until surgical correction at the age of 7. At
SC R
the age of 25 the patient showed a mean pulmonary arterial pressure of 22mmHg and thus borderline PAH in right heart catheter. In the outpatient clinic no contraindication against pregnancy was reported although the patient had severe regurgitation of the pulmonic valve but sPAP was not elevated in
NU
echocardiography. During pregnancy the patient was increasingly limited in physical activities and did gain weight with ankle and leg swelling. The patient had to stay in hospital after completion of the 26th
MA
week of pregnancy and had a maximum sPAP of 80-90mmHg derived from echocardiography. As a consequence diuretics were administered and due to placental insufficiency, Caesarean section was
D
performed in the 32th week of pregnancy because of constant worsening of the fetus. Afterwards, the
TE
patient had a prolonged intensive care unit stay with combination therapy of ERA and PPI5 and still elevated sPAP with 60mmHg. Right and left heart catheterization after birth did show near to systemic
CE P
pressure in the pulmonary arteries, elevated left ventricular filling pressures with 35mmHg and a central venous pressure of 30mmHg. Following a long hospital stay the patient is on PAH combination
AC
therapy and is listed for heart and lung transplantation. (Figure 1 shows the echocardiography in each case before and after pregnancy). Pulmonary-arterial hypertension has a plethora of facets which have to be accounted for in congenital and especially cyanotic heart disease [1, 3]. The cases illustrate the difficult circumstances which have to be overcome by the patients and also the treating physicians. Patients which were advised not to become pregnant did decide to do so and were treated as high-risk pregnancy in a multidisciplinary team and delivered successfully. However, on the contrary one patient with a supposed low-risk pregnancy in cyanotic congenital heart disease had an extreme adverse course, illustrating the potential risk of remodeling of the right ventricle during pregnancy [5].
Conflicts of Interest: None
ACCEPTED MANUSCRIPT
References
AC
CE P
TE
D
MA
NU
SC R
IP
T
[1] Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart JM, et al. 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. 2011;32:3147-97. [2] Schmaltz AA, Neudorf U, Winkler UH. Outcome of pregnancy in women with congenital heart disease. Cardiol Young. 1999;9:88-96. [3] Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31:2915-57. [4] Niwa K, Perloff JK, Kaplan S, Child JS, Miner PD. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol. 1999;34:223-32. [5] Egidy Assenza G, Cassater D, Landzberg M, Geva T, Schreier J, Graham D, et al. The effects of pregnancy on right ventricular remodeling in women with repaired tetralogy of Fallot. Int J Cardiol. 2013;168:1847-52.
ACCEPTED MANUSCRIPT
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D
MA
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Figure 1 Echocardiography in case 1(A),2(B) and 3(C)
