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2022, Number S5

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Cardiovasc Metab Sci 2022; 33 (S5)

Congenital heart disease in women

Romero-Rivera, Ivan1; Yáñez-Gutiérrez, Lucelli2; Meza-Méndez, Yolimar3; Morr-García, Igor4
Full text How to cite this article 10.35366/108051

DOI

DOI: 10.35366/108051
URL: https://dx.doi.org/10.35366/108051

Language: English
References: 11
Page: s454-457
PDF size: 164.11 Kb.


Key words:

No keywords





INTRODUCTION

Advances in diagnosis, anesthesia, intensive care, and surgery have improved the evolution of congenital heart disease (CHD). Sixty years ago, only 25% survived beyond the first year of life. Currently, more than 95% survived to adulthood. It is estimated that 1/150 adults in the United States of America (USA) have different types of CHD, from subclinical forms of the bicuspid aortic valve to severe conditions; there are approximately 10 million adults with CHD in the world.

Khairy et al. analyze mortality in 71,686 patients. They observed that in 2004-2005 mortality was similar to that of the general population, quite different from the observed in 1987-1988, where high mortality was observed in the first year of life.

The estimated number of adults with CHD in the USA was 1,444,500 in 2016, a 63% increase in the estimated population with CHD since 2000. The prevalence in women was 6/1,000 and in men 3.8/1,000.

According to DiNardo, adults with CHD may: 1) have simple or complex lesions that allow survival and are seen for the first time; 2) have previous palliative procedures waiting for a new procedure; 3) be awaiting early reoperation; 4) be awaiting surgery for residual lesions; 5) be awaiting transplant; 6) also acquired heart disease. These heart diseases can be classified anatomically as type I) simple (≈ 45%), II) of moderate complexity (≈ 40%), and III) of great complexity (≈ 15%) (Figure 1).

To facilitate the approach, the AHA/ACC created a functional classification: A, B, C, and D, similar to that of the NYHA for heart failure (HF), but with characteristics typical of adult CHD, indicating that patients in the classifications I B-D; II A-D and III A-D must be explored by a specialist with experience in adult congenital heart disease.



PREGNANCY

Data from the multicenter ROPAC Registry, with 57.4% of pregnant women with CHD, showed maternal mortality of 0.6% in the total group and 0.2% in those with CHD, much higher than expected (0.007% in average pregnant women).

Pre-pregnancy data, such as NYHA HF or CF signs > II, left ventricular ejection fraction < 40%, modified World Health Organization (WHO) class IV, and use of anticoagulation, were cited as predictors of HF or mortality during pregnancy.

In a review of 48 studies performed between 1983 and 2006, including 2,491 pregnant women with CHD, 34.2% have obstetric complications (2.2% embolic events, 8.7% hypertensive disease, 11% early delivery, and 3.9% premature rupture of the membranes), 11.7% of maternal cardiac complications (4.5% of arrhythmia, 4.8% of heart failure and 1.9% of other pathologies such as heart attack, stroke, etc.) and 31.4% of fetal complications among which the most important was preterm birth. CHD recurrence in children was 3.5%, and infective endocarditis (IE) was a rare event (0.5%).

The number of pregnancies in patients with CHD increases rapidly. Data from the US National Inpatient Sample from 1998-2007 show that the number of deliveries in patients with CHD increased by 34.9%, compared to an increase of 21.3% in the general population.



THE CARE OF A PREGNANT WOMAN WITH CHD INVOLVES:

  • 1. Preconception: women should be assessed before planning a pregnancy using risk stratification scales (ZAHARA, CARPREG, and WHO). The modified WHO risk stratification should be used initially (Table 1).
  • 2. During pregnancy: Multidisciplinary care to diagnose and treat possible complications. Guide the use of cardiovascular drugs with effects on the fetus.
  • 3. In childbirth: except for some heart diseases, the indication for delivery is obstetric. It is essential to prevent complications related to pain, anxiety, and massive venous return when decompressing the pregnant uterus or using thromboprophylaxis.
  • 4. Puerperium: divided into immediate (24 hours), mediate (7 days), and late (42 days), will be followed up to one year after delivery or interruption of pregnancy. Guiding family planning without interfering with the use of cardiovascular drugs. The physiological changes of pregnancy tend to normalize 45 days after delivery, requiring cardiovascular evaluation at this time.



COMPLICATIONS

Patients with CHD generally use medication for life and require multiple surgeries and hospital admissions; they can present a higher number of complications or mortality during pregnancy, severe arrhythmias and heart failure, and reduced life expectancy. We cannot forget acquired diseases, such as systemic arterial hypertension, acute myocardial infarction, and cerebral vascular accident.

IE is more frequent than in the general population and more severe in prosthetic valves, including those with percutaneous implantation, valve repair with a prosthetic ring, previous IE, any cyanotic CHD or repaired with prosthetic material, up to 6 months after the procedure or permanent if a residual shunt or valvular insufficiency persists.

Antibiotic prophylaxis is recommended in patients at high risk for IE, even during childbirth, without consensus on the preventive use of antibiotics in all women with CHD.

Bleeding diathesis and thrombosis are frequent complications, and their treatment and prophylaxis contribute to survival. Spontaneous bleeding is mild and self-limited (dental, epistaxis, bruising, menorrhagia). Hemoptysis is the external manifestation of intrapulmonary hemorrhage, common in Eisenmenger syndrome.

Concerning thrombosis, the associated risk factors are female gender, oxygen desaturation, senility, biventricular dysfunction, dilatation of pulmonary arteries, and post-surgical complications such as Fontan, in which there may be total or partial thrombotic obstruction of the shunt cavopulmonary. Its treatment includes thrombectomy, percutaneous angioplasty (stent), surgery, and anticoagulant therapy or thrombolysis.

Strokes are frequent and related to thromboembolic events, microcytosis, endothelial dysfunction, and paradoxical emboli due to endocavitary electrodes and catheters. Anticoagulation is not routinely indicated in patients with Pulmonary Hypertension, and its prescription is individualized, as in the case of mechanical valves, vascular prostheses, supraventricular arrhythmias, presence of thrombosis or pulmonary embolism, provided that the bleeding risk is low.


REFERENCES

  1. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JI et al. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol. 2001; 37 (5): 1170-1175.

  2. Warnes CA. The adult with congenital heart disease: born to be bad? J Am Coll Cardiol. 2005; 46 (1): 1-8.

  3. Khairy P, Ionescu-Ittu R, Mackie AS, Abrahamowicz M, Pilote L, Marelli AJ. Changing mortality in congenital heart disease. J Am Coll Cardiol. 2010; 56 (14): 1149-1157.

  4. Gilboa SM, Devine OJ, Kucik JE, Oster ME, Riehle-Colarusso T, Nembhard WN et al. Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010. Circulation. 2016; 134 (2): 101-109.

  5. DiNardo JA. Grown-up congenital heart (GUCH) disease: an evolving global challenge. Ann Card Anaesth. 2008; 11 (1): 3-5.

  6. Roos-Hesselink J, Baris L, Johnson M, De Backer J, Otto C, Marelli A et al. Pregnancy outcomes in women with cardiovascular disease: evolving trends over ten years in the ESC Registry Of Pregnancy And Cardiac disease (ROPAC). Eur Heart J. 2019; 40 (47): 3848-3855.

  7. Opotowsky AR, Siddiqi OK, D'Souza B, Webb GD, Fernandes SM, Landzberg MJ. Maternal cardiovascular events during childbirth among women with congenital heart disease. Heart. 2012; 98: 145-151.

  8. Drenthen W, Pieper PG, Roos-Hesselink JW, van Lottum WA, Voors AA, Mulder BJ et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol. 2007; 49: 2303-2311.

  9. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, Blomström-Lundqvist C, Cífková R, De Bonis M et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018; 39 (34): 3165-3241.

  10. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139 (14): e698-e800.

  11. Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021; 42 (6): 563-645.



AFFILIATIONS

1 Federal University of Alagoas; Santa Casa de Misericórdia Hospital in Maceió, Brazil.

2 Cardiology Hospital. XXI Century National Medical Center, Mexico.

3 Maracay Polyclinic, Aragua, Venezuela.

4 Professor of Medicine at the Faculty of Health Sciences of the University of Carabobo and the Central University of Venezuela, Venezuela.



CORRESPONDENCE

Ivan Romero-Rivera. E-mail: irrivera@uol.com.br


Figure 1
Table 1

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Cardiovasc Metab Sci . 2022;33