Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals
<i>Background and Objectives</i>: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when a...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-03-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/58/4/476 |
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| author | Virginija Rudienė Lina Kaplerienė Dovilė Jančauskaitė Emilija Meškėnė Eglė Palevičiūtė Monika Laukytė-Slėnienė Diana Gasiūnaitė Diana Ramašauskaitė Elena Jurevičienė Lina Gumbienė |
| author_facet | Virginija Rudienė Lina Kaplerienė Dovilė Jančauskaitė Emilija Meškėnė Eglė Palevičiūtė Monika Laukytė-Slėnienė Diana Gasiūnaitė Diana Ramašauskaitė Elena Jurevičienė Lina Gumbienė |
| author_sort | Virginija Rudienė |
| collection | DOAJ |
| description | <i>Background and Objectives</i>: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients’ well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. <i>Materials and Methods</i>: The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. <i>Results</i>: Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. <i>Conclusions</i>: On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes. |
| first_indexed | 2024-03-09T13:20:01Z |
| format | Article |
| id | doaj.art-06290307d2674ac89fcf3568a4d72f12 |
| institution | Directory Open Access Journal |
| issn | 1010-660X 1648-9144 |
| language | English |
| last_indexed | 2024-03-09T13:20:01Z |
| publishDate | 2022-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-06290307d2674ac89fcf3568a4d72f122023-11-30T21:30:10ZengMDPI AGMedicina1010-660X1648-91442022-03-0158447610.3390/medicina58040476Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare ProfessionalsVirginija Rudienė0Lina Kaplerienė1Dovilė Jančauskaitė2Emilija Meškėnė3Eglė Palevičiūtė4Monika Laukytė-Slėnienė5Diana Gasiūnaitė6Diana Ramašauskaitė7Elena Jurevičienė8Lina Gumbienė9Clinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaClinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaClinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaClinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaClinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaClinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaCentre of Anaesthesiology, Intensive Therapy and Pain Management, Faculty of Medicine, Vilnius University, LT-08661 Vilnius, LithuaniaFaculty of Medicine, Vilnius University, LT-03101 Vilnius, LithuaniaFaculty of Medicine, Vilnius University, LT-03101 Vilnius, LithuaniaClinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, Lithuania<i>Background and Objectives</i>: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients’ well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. <i>Materials and Methods</i>: The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. <i>Results</i>: Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. <i>Conclusions</i>: On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes.https://www.mdpi.com/1648-9144/58/4/476pulmonary arterial hypertensioncongenital heart diseasepregnancyrare diseasesmultidisciplinary team |
| spellingShingle | Virginija Rudienė Lina Kaplerienė Dovilė Jančauskaitė Emilija Meškėnė Eglė Palevičiūtė Monika Laukytė-Slėnienė Diana Gasiūnaitė Diana Ramašauskaitė Elena Jurevičienė Lina Gumbienė Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals Medicina pulmonary arterial hypertension congenital heart disease pregnancy rare diseases multidisciplinary team |
| title | Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals |
| title_full | Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals |
| title_fullStr | Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals |
| title_full_unstemmed | Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals |
| title_short | Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals |
| title_sort | pregnancy in congenital heart disease complicated by pulmonary arterial hypertension a challenging issue for the pregnant woman the foetus and healthcare professionals |
| topic | pulmonary arterial hypertension congenital heart disease pregnancy rare diseases multidisciplinary team |
| url | https://www.mdpi.com/1648-9144/58/4/476 |
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