Cardiovascular outcomes and aortic growth in pregnant women with Turner syndrome: data from the European Society of Cardiology EURObservational Research Programme Registry of Pregnancy and Cardiac Disease (ROPAC) III - Scorecard - MDSpire

Cardiovascular outcomes and aortic growth in pregnant women with Turner syndrome: data from the European Society of Cardiology EURObservational Research Programme Registry of Pregnancy and Cardiac Disease (ROPAC) III

  • By

  • Johanna A van der Zande

  • Jasmin Grewal

  • Arie Franx

  • Joanna Hlebowicz

  • Laurent Demulier

  • Thelma C Konings

  • Abdulhalim J Kinsara

  • Julius C Mwita

  • Laurence Bal-Theoleyre

  • Wael Al Mahmeed

  • Lidija McGrath

  • Roger Hall

  • Mark R Johnson

  • Jolien W Roos-Hesselink

  • ROPAC investigators

  • R Ferrari

  • A Alonso

  • J Bax

  • C Blomström-Lundqvist

  • S Gielen

  • P Lancellotti

  • A P Maggioni

  • N Maniadakis

  • F Pinto

  • F Ruschitzka

  • L Tavazzi

  • P Vardas

  • F Weidinger

  • U Zeymer

  • A Vahanian

  • A Budaj

  • N Dagres

  • N Danchin

  • V Delgado

  • J Emberson

  • O Friberg

  • C P Gale

  • G Heyndrickx

  • B Iung

  • S James

  • A P Kappetein

  • A P Maggioni

  • N Maniadakis

  • K V Nagy

  • G Parati

  • A-S Petronio

  • M Pietila

  • E Prescott

  • F Ruschitzka

  • F Van de Werf

  • F Weidinger

  • U Zeymer

  • C P Gale

  • B Beleslin

  • A Budaj

  • O Chioncel

  • N Dagres

  • N Danchin

  • J Emberson

  • D Erlinge

  • M Glikson

  • A Gray

  • M Kayikcioglu

  • A P Maggioni

  • K V Nagy

  • A Nedoshivin

  • A-P Petronio

  • J W Roos-Hesselink

  • L Wallentin

  • U Zeymer

  • B A Popescu

  • D Adlam

  • A L P Caforio

  • D Capodanno

  • M Dweck

  • D Erlinge

  • M Glikson

  • J Hausleiter

  • B Iung

  • M Kayikcioglu

  • P Ludman

  • L Lund

  • A P Maggioni

  • S Matskeplishvili

  • B Meder

  • K V Nagy

  • A Nedoshivin

  • D Neglia

  • A A Pasquet

  • J W Roos-Hesselink

  • F J Rossello

  • S M Shaheen

  • A Torbica

  • Jolien Roos-Hesselink

  • Roger Hall

  • William Parsonage

  • Werner Budts

  • Julie De Backer

  • Jasmin Grewal

  • Ariane Marelli

  • Guillaume Jondeau

  • Mark Johnson

  • Catherine Otto

  • Karen Sliwa

  • Aldo Maggioni

  • K Vardanyan

  • A Melkonyan

  • H Lachikyan

  • K Hakobyan

  • M Mazmanian

  • H Hayrapetyan

  • A Tavaracyan

  • H Poghosyan

  • R Hovhannisyan

  • S Sahakyan

  • S Martirosyan

  • J Harris

  • A Pasquet

  • M Morissens

  • T Besse-Hammer

  • B Dumoulin

  • J De Backer

  • L Campens

  • L Demulier

  • M De Hosson

  • W Budts

  • A Van de Bruaene

  • A Rampelberg

  • E Troost

  • L Roggen

  • P De Meester

  • J C Mwita

  • E Tefera

  • L Kontle

  • A Marelli

  • I Malhamé

  • J Grewal

  • M Janzen

  • P A Román Rubio

  • R Vasallo Peraza

  • G Vázquez Hernández

  • J E Pérez Torga

  • Y Gil Jiménez

  • M Meluzá Martín

  • R Almaleh

  • G Youssef

  • K Sorour

  • S Abebe

  • D Mekonnen

  • C Fekadu

  • D Yadeta

  • S Dupuis-Girod

  • L Delagrange

  • M Richardson

  • L Ghesquiere

  • O Domanski

  • M Gonzalez Estevez

  • Y Ould Hamoud

  • S Gautier

  • L Marsili

  • L Bal-Theoleyre

  • S Palazzolo

  • M Ladouceur

  • G Jondeau

  • A Bourgeois Moine

  • L Eliaou

  • O Milleron

  • M Tchitchinadze

  • Y Dulac

  • C Karsenty

  • N Souletie

  • F Bajanca

  • C Rickers

  • S Blankenberg

  • C Sinning

  • C Magnussen

  • E Zengin

  • G Mueller

  • R Schnabel

  • Y Von Kodolitsch

  • R Kozlik-Feldmann

  • H Baumgartner

  • R Schmidt

  • A Hellige

  • A Rietkötter

  • M Spartalis

  • A A Frogoudaki

  • A Arvanitaki

  • A Baroutidou

  • G Giannakoulas

  • C Karvounis

  • J P Gnanaraj

  • A P Steaphen

  • T Ethirajan

  • K Kannan

  • V Subramanian

  • A Surendran

  • J Gnanasekaran

  • V Natarajalingam

  • S Balasubramani

  • H Ali Farhan

  • I F Yaseen

  • E Mariucci

  • C Ciuca

  • F Marchi

  • G Benedetti

  • M Baroni

  • P Festa

  • A Parlanti

  • G Scognamiglio

  • F Fusco

  • B Sarubbi

  • M Merlo

  • B D'Agata Mottolese

  • C Carriere

  • G Sinagra

  • M Bobbo

  • F Ramani

  • F M Comoglio

  • R Bordese

  • A Pagano

  • N Montali

  • V Donvito

  • C A Remolif

  • F Petey

  • B Bouma

  • S Chamuleau

  • D Robbers-Visser

  • T Konings

  • H Dronkert

  • D Segers

  • R Van Kimmenade

  • H Van Der Zwaan

  • G Tjalling Sieswerda

  • A Evers

  • T Schaap

  • K Bano

  • H Yasmeen

  • K Amir

  • N Patel

  • P Akhter

  • R Khan

  • A Shakeel

  • S Mahar

  • S Habib

  • M Lelonek

  • P Hoffman

  • M Lipczynska

  • L De Sousa

  • V Ferreira

  • T Mano

  • M Selas

  • R Cruz Ferreira

  • E Shlyakhto

  • O Irtyuga

  • G Sefieva

  • K Malikov

  • T Pervunina

  • U Shadrina

  • A Kinsara

  • D Galzerano

  • H Al Sergani

  • W Kurdi

  • N Kholaif

  • O Vriz

  • A Alhamshari

  • A Alsaigh

  • O Ahmad

  • S Alzaher

  • B Alamro

  • K Sliwa

  • F Jakoet-Bassier

  • L Galian-Gay

  • A Pijuan-Domenech

  • B Miranda-Barrio

  • B Gordon

  • E Furenäs

  • J Hlebowicz

  • F Wedlund

  • E Nagy

  • E Mattsson

  • M Majczuk Sennstrom

  • P Sörensson

  • C Christersson

  • A Lutvica

  • B Jönelid

  • T Achter

  • K Junus

  • H Gärdesten Wall

  • M Andreasson

  • D Tobler

  • J Bouchardy

  • F Brand

  • C Blanche

  • J Bouchardy

  • T Rutz

  • F Brand

  • U Canpolat

  • Y Z Sener

  • N Ozer

  • E Ayduk Gövdeli

  • Z Bugra

  • B Umman

  • P Karaca Özer

  • D Baykiz

  • D Mutlu

  • H Yalman

  • B Kilickiran Avci

  • S Catirli Enar

  • O Batukan Esen

  • D Oksen

  • V Lazoryshynets

  • S Siromakha

  • Y Davydova

  • A Limanska

  • I Zinovchyk

  • V Kravchenko

  • B Kravchuk

  • O Kravets

  • N Volkova

  • O Mazur

  • O Beregovyi

  • B T Salih

  • W A R Al Mahmeed

  • S Wani

  • F S Mohamed Farook

  • G Al Mansoori

  • S Prakash

  • R Afifi

  • D Milewicz

  • A Cecchi

  • G Wells

  • D Sparks

  • W Wagner

  • C Bigelow

  • L Colicchia

  • T Jentink

  • M Loichinger

  • R Saxena

  • W Wunderlich

  • C Longtin

  • P Klopper

  • R Gobar

  • J Chou

  • K Campbell

  • R Elder

  • D Halpern

  • A Hausvater

  • H Reynolds

  • N Bhalla

  • A Small

  • J Feinberg

  • P Panday

  • J Awerbach

  • J Porche

  • S Stack

  • L McGrath

  • A Khan

  • E Pare

  • P Woods

  • C Broberg

  • K Gibbins

  • K Brookfield

  • M Al-Sadawi

  • A Cove

  • N Mann

  • September 8, 2025

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Clinical Scorecard: Cardiovascular and Aortic Growth Outcomes During Pregnancy in Women with Turner Syndrome: Insights from the ROPAC III Registry of the European Society of Cardiology

At a Glance

CategoryDetail
ConditionTurner syndrome with cardiovascular involvement during pregnancy
Key MechanismsAortic dilatation and growth, bicuspid aortic valve, hypertension, and risk of aortic dissection
Target PopulationPregnant women diagnosed with Turner syndrome prior to pregnancy
Care SettingSpecialized centers for pregnancy and cardiac disease monitoring

Key Highlights

  • No maternal mortality, aortic dissection, or other cardiac events occurred in 47 pregnancies studied.
  • Significant aortic growth at the sinus of Valsalva occurred during pregnancy but was reversible postpartum.
  • Hypertensive disorders occurred in 26% of pregnancies and require careful management.

Guideline-Based Recommendations

Diagnosis

  • Pre-pregnancy diagnosis of Turner syndrome with cardiac evaluation including aortic size index (ASI).
  • Assessment of risk factors such as bicuspid aortic valve, aortic dilatation, hypertension, and coarctation of the aorta.

Management

  • Pregnancy contraindicated if ASI >25 mm/m2 (ESC guidelines).
  • Close monitoring recommended if ASI between 20 and 25 mm/m2, especially with additional risk factors.
  • Consideration of assisted reproductive technology (ART) risks, including higher Caesarean section rates and earlier delivery.
  • Management of hypertensive disorders during pregnancy is essential.

Monitoring & Follow-up

  • Regular imaging to monitor aortic diameter and growth during pregnancy.
  • Postpartum follow-up to assess reversibility of aortic dilatation.
  • Monitoring for hypertensive disorders and cardiac complications throughout pregnancy.

Risks

  • Increased risk of aortic dissection associated with BAV, hypertension, and coarctation.
  • Hypertensive disorders are common and may complicate pregnancy.
  • ART pregnancies have higher rates of Caesarean delivery and earlier gestational age at delivery.

Patient & Prescribing Data

Pregnant women with Turner syndrome, including those conceiving spontaneously or via ART

No maternal mortality or aortic dissection observed; hypertensive disorders require management; ART associated with higher Caesarean rates and earlier delivery.

Clinical Best Practices

  • Pre-pregnancy cardiovascular risk stratification including ASI measurement.
  • Multidisciplinary care involving cardiology and obstetrics in expert centers.
  • Close surveillance of aortic dimensions during pregnancy with imaging.
  • Management and prevention of hypertensive disorders during pregnancy.
  • Counseling regarding risks associated with ART and pregnancy outcomes.

References

Original Source(s)

Cardiovascular outcomes and aortic growth in pregnant women with Turner syndrome: data from the European Society of Cardiology EURObservational Research Programme Registry of Pregnancy and Cardiac Disease (ROPAC) III

  • by Johanna A van der Zande, Jasmin Grewal, Arie Franx, Joanna Hlebowicz, Laurent Demulier, Thelma C Konings, Abdulhalim J Kinsara, Julius C Mwita, Laurence Bal-Theoleyre, Wael Al Mahmeed, Lidija McGrath, Roger Hall, Mark R Johnson, Jolien W Roos-Hesselink, ROPAC investigators, R Ferrari, A Alonso, J Bax, C Blomström-Lundqvist, S Gielen, P Lancellotti, A P Maggioni, N Maniadakis, F Pinto, F Ruschitzka, L Tavazzi, P Vardas, F Weidinger, U Zeymer, A Vahanian, A Budaj, N Dagres, N Danchin, V Delgado, J Emberson, O Friberg, C P Gale, G Heyndrickx, B Iung, S James, A P Kappetein, A P Maggioni, N Maniadakis, K V Nagy, G Parati, A-S Petronio, M Pietila, E Prescott, F Ruschitzka, F Van de Werf, F Weidinger, U Zeymer, C P Gale, B Beleslin, A Budaj, O Chioncel, N Dagres, N Danchin, J Emberson, D Erlinge, M Glikson, A Gray, M Kayikcioglu, A P Maggioni, K V Nagy, A Nedoshivin, A-P Petronio, J W Roos-Hesselink, L Wallentin, U Zeymer, B A Popescu, D Adlam, A L P Caforio, D Capodanno, M Dweck, D Erlinge, M Glikson, J Hausleiter, B Iung, M Kayikcioglu, P Ludman, L Lund, A P Maggioni, S Matskeplishvili, B Meder, K V Nagy, A Nedoshivin, D Neglia, A A Pasquet, J W Roos-Hesselink, F J Rossello, S M Shaheen, A Torbica, Jolien Roos-Hesselink, Roger Hall, William Parsonage, Werner Budts, Julie De Backer, Jasmin Grewal, Ariane Marelli, Guillaume Jondeau, Mark Johnson, Catherine Otto, Karen Sliwa, Aldo Maggioni, K Vardanyan, A Melkonyan, H Lachikyan, K Hakobyan, M Mazmanian, H Hayrapetyan, A Tavaracyan, H Poghosyan, R Hovhannisyan, S Sahakyan, S Martirosyan, J Harris, A Pasquet, M Morissens, T Besse-Hammer, B Dumoulin, J De Backer, L Campens, L Demulier, M De Hosson, W Budts, A Van de Bruaene, A Rampelberg, E Troost, L Roggen, P De Meester, J C Mwita, E Tefera, L Kontle, A Marelli, I Malhamé, J Grewal, M Janzen, P A Román Rubio, R Vasallo Peraza, G Vázquez Hernández, J E Pérez Torga, Y Gil Jiménez, M Meluzá Martín, R Almaleh, G Youssef, K Sorour, S Abebe, D Mekonnen, C Fekadu, D Yadeta, S Dupuis-Girod, L Delagrange, M Richardson, L Ghesquiere, O Domanski, M Gonzalez Estevez, Y Ould Hamoud, S Gautier, L Marsili, L Bal-Theoleyre, S Palazzolo, M Ladouceur, G Jondeau, A Bourgeois Moine, L Eliaou, O Milleron, M Tchitchinadze, Y Dulac, C Karsenty, N Souletie, F Bajanca, C Rickers, S Blankenberg, C Sinning, C Magnussen, E Zengin, G Mueller, R Schnabel, Y Von Kodolitsch, R Kozlik-Feldmann, H Baumgartner, R Schmidt, A Hellige, A Rietkötter, M Spartalis, A A Frogoudaki, A Arvanitaki, A Baroutidou, G Giannakoulas, C Karvounis, J P Gnanaraj, A P Steaphen, T Ethirajan, K Kannan, V Subramanian, A Surendran, J Gnanasekaran, V Natarajalingam, S Balasubramani, H Ali Farhan, I F Yaseen, E Mariucci, C Ciuca, F Marchi, G Benedetti, M Baroni, P Festa, A Parlanti, G Scognamiglio, F Fusco, B Sarubbi, M Merlo, B D'Agata Mottolese, C Carriere, G Sinagra, M Bobbo, F Ramani, F M Comoglio, R Bordese, A Pagano, N Montali, V Donvito, C A Remolif, F Petey, B Bouma, S Chamuleau, D Robbers-Visser, T Konings, H Dronkert, D Segers, R Van Kimmenade, H Van Der Zwaan, G Tjalling Sieswerda, A Evers, T Schaap, K Bano, H Yasmeen, K Amir, N Patel, P Akhter, R Khan, A Shakeel, S Mahar, S Habib, M Lelonek, P Hoffman, M Lipczynska, L De Sousa, V Ferreira, T Mano, M Selas, R Cruz Ferreira, E Shlyakhto, O Irtyuga, G Sefieva, K Malikov, T Pervunina, U Shadrina, A Kinsara, D Galzerano, H Al Sergani, W Kurdi, N Kholaif, O Vriz, A Alhamshari, A Alsaigh, O Ahmad, S Alzaher, B Alamro, K Sliwa, F Jakoet-Bassier, L Galian-Gay, A Pijuan-Domenech, B Miranda-Barrio, B Gordon, E Furenäs, J Hlebowicz, F Wedlund, E Nagy, E Mattsson, M Majczuk Sennstrom, P Sörensson, C Christersson, A Lutvica, B Jönelid, T Achter, K Junus, H Gärdesten Wall, M Andreasson, D Tobler, J Bouchardy, F Brand, C Blanche, J Bouchardy, T Rutz, F Brand, U Canpolat, Y Z Sener, N Ozer, E Ayduk Gövdeli, Z Bugra, B Umman, P Karaca Özer, D Baykiz, D Mutlu, H Yalman, B Kilickiran Avci, S Catirli Enar, O Batukan Esen, D Oksen, V Lazoryshynets, S Siromakha, Y Davydova, A Limanska, I Zinovchyk, V Kravchenko, B Kravchuk, O Kravets, N Volkova, O Mazur, O Beregovyi, B T Salih, W A R Al Mahmeed, S Wani, F S Mohamed Farook, G Al Mansoori, S Prakash, R Afifi, D Milewicz, A Cecchi, G Wells, D Sparks, W Wagner, C Bigelow, L Colicchia, T Jentink, M Loichinger, R Saxena, W Wunderlich, C Longtin, P Klopper, R Gobar, J Chou, K Campbell, R Elder, D Halpern, A Hausvater, H Reynolds, N Bhalla, A Small, J Feinberg, P Panday, J Awerbach, J Porche, S Stack, L McGrath, A Khan, E Pare, P Woods, C Broberg, K Gibbins, K Brookfield, M Al-Sadawi, A Cove, N Mann

  • September 8, 2025

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