Absent Pulmonary Valve Syndrome – Special Prenatal Care and Early Surgery in Obstetric/Cardiac Center - A New Hope for Postnatal Survivors?

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Introduction: APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 - 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality. Material and methods: In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome. Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05). Conclusions: There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.

1. Slodki M, Szymkiewicz-Dangel J, Tobota Z, Seligman NS, Weiner S, Respondek-Liberska M. The Polish National Registry for Fetal Cardiac Pathology: organization, diagnoses, management, educational aspects and telemedicine endeavors. Prenat Diagn. 2012; 32: 456-60

2. Kordjalik P, Radzymińska Chruściel B, Słodki M, Włoch A, Szymkiewicz-Dangel J, Respondek-Liberska M, Tobota Z.: The Polish National Registry for Fetal Cardiac Pathology (www.orpkp.pl) - selected data analysis for 2013 and 2014 and comparison with data from 2004 to 2012. Prenat Cardio. 2015; 5: 6-12

3. Respondek-Liberska M.: Prenatal Cardiology for Obstetricians and Pediatric Cardiologists. Publishing House: Czelej, Lublin 2006

4. Słodki M, Respondek-Liberska M.: Proposal of screening fetal heart examination form granted by Polish Ministry of Health Program Kardio-Prenatal 2008. Ginekol Pol. 2009; 80: 466-70

5. Słodki M, Respondek-Liberska M, Pruetz JD, Donofrio MT.: Fetal cardiology: changing the definition of critical heart disease in the newborn. J Perinatol. 2016; 36: 575-80

6. Strzelecka I, Słodki M, Płużańska J, Moszura T, Węgrzynowski J, Respondek-Liberska M.: Routine third trimester fetal cardiac evaluation: time for consideration. Prenat Cardio. 2015; 5:18-23

7. Axt-Fliedner R, Kurkevych A, Slodki M, Respondek-Liberska M, Zych-Krekora K, Stressig R, Ritgen J, Rizzo G, Krapp M, de Catte L, Mielke G, Bosselmann S, Meyer-Wittkopf M, Kawecki A, Wolter A, Mamalis M, Enzensberger C.: International Prenatal Cardiology Collaboration Group. Absent pulmonary valve syndrome - diagnosis, associations, and outcome in 71 prenatally diagnosed cases. Prenat Diagn. 2017; 37: 812-819

8. Chevers N.: Recherches maladies arteres pulmonaires. Arch Gen Med 1847; 15: 488-508

9. Fouron JC, Sahn DJ, Bender R, Block R, Schneider H, Fromberger P, Hagen-Ansert S, Daily PO. Prenatal diagnosis and circulatory characteristics in tetralogy of Fallot with absent pulmonary valve. Am J Cardiol 1989; 64: 547-549

10. Nawara A, Radzymińska-Chruściel B, Respondek Liberska M, Januszewska K, Malec E.: First trimester diagnosis of Absent Pulmonary Valve (APV): a case report of prenatal treatment with a review of literature and data from The Polish National Registry of Fetal Cardiac Pathology, Prenat Cadio 2014; 4: 18-2

11. Calder AL, Brandt PW, Barratt-Boyes BG, Neutz JM.: Variant of tetralogy of Fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta, Am. J. Cardiol. 1980; 46: 106

12. Snir E, de Leval MR, Elliott MJ, Stark J.: Current surgical technique to repair Fallot’s tetralogy with absent pulmonary valve syndrome. Ann. Thorac. Surg. 1991; 51: 97

13. Razavi RS, Sharland GK, Simpson JM.: Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome. Am J Cardiol 2003; 91: 429-432

14. Galindo A, Gutiérrez-Larraya, F, Martínez JM del Rio, Grañeras A, Velasco JM, Puerto B and Gratacos E.: Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve. Ultrasound Obstet Gynecol 2006; 28: 32-39

15. Volpe P, Paladini, D, Marasini M, Buonadonna AL, Russo MG, Caruso G, Marzullo A, Arciprete, P, Martinelli, P and Gentile M.: Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol 2004; 24: 623-628

16. Wertaschnigg, D, Jaeggi M, Chitayat D, Shannon P, Ryan G, Thompson M, Yoo SJ and Jaeggi, E.: Prenatal diagnosis and outcome of absent pulmonary valve syndrome: contemporary single-center experience and review of the literature. Ultrasound Obstet Gynecol 2013;41: 162-167

17. Szwast A, Tian Z, McCann M, Soffer D, Combs J, Donahhue D, Rychik J.: Anatomic Variability and Outcome in Prenatally Diagnosed Absent Pulmonary Valve Syndrome. Ann Thorac Surg 2014; 98: 152-158

18. Yong M, Yim D, Brizard Ch,Robertson T, Bullock A, d’Udekem Y, Konstantinov I.: Long-Term Outcomes of Patients With Absent Pulmonary Valve Syndrome: 38 Years of Experience. Ann Thorac Surg. 2014; 97: 1671-1677

19. Azancot-Benisty A, Jacqz-Aigrain E, Guirgis N, Decrepy A, Oury JF, Blot P.: Clinical and pharmacologic study of fetal supraventricular tachyarrhythmias. J Pediatr. 1992; 121: 608-613

20. Strzelecka I, Respondek-Liberska M, Słodki M, Zych-Krekora K. Cuneo B. :Transplacental Digoxin Treatment In Prenatal Cardiac Problems In Singleton Pregnancies - Meta Analysis (Based On Literature: 1992-2015). Prenat Cardio 2016; 6: 67-74

21. Patel D, Cuneo B, Viesca R, Rassanan J, Leshko J, Huhta J.: Digoxin for the treatment of fetal congestive heart failure with sinus rhythm assessed by cardiovascular profile score. J Matern Fetal Neonatal Med. 2008; 21: 477-482

22. Respondek-Liberska M, Jarosik K, Janiak K, Sysa A, Kowalska-Koprek U, Wilczyński J, Stańczyk J.: Outcome of fetuses with tachyarrhythmias > 200/min - bicenter Łódź study. Pol Przegl Kardiol 2006; 8: 35-39

23. Kleinman CS, Donnerstein RL, DeVore GR, Jaffe CC, Lynch DC, Berkowitz RL, Talner NS, Hobbins JC.: Fetal Echocardiography for Evaluation of in Utero Congestive Heart Failure. N Engl J Med 1982; 306: 568-575

24. Eckersley L, Sadler L, Parry E, Finucane K, Gentles TL.: Timing of diagnosis affects mortality in critical congenital heart disease, Archives of Disease in Childhood 2016; 101: 516-520

25. Jowett V, Sankaran, S, Rollings S, Hall, R., Kyle, P, Sharland G.: Foetal congenital heart disease: Obstetric management and time to first cardiac intervention in babies delivered at a tertiary centre. Cardiology in the Young, 2014; 24: 494-502

26. Sierra J,, Christenson JT,, Lahlaidi NH,, Beghetti M,, Kalangos A.:. Right Ventricular Outflow Tract Reconstruction: What Conduit to Use? Homograft or Contegra? Annals of Thoracic Surgery 2007; 84: 606-611

27. Chen JM, Glickstein JS, Margossian R, Mercando ML, Hellenbrand WM, Mosca RS, Quaegebeur JM.: Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome. J Thorac Cardiovasc Surg 2006; 132: 1099-1104

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