Successful pregnancy in ASD patient – complicated by severe Pulmonary Arterial Hypertension

Ramya1, N. Suchitra2, T. Joseph3

1MEM Resident, Kauvery Hospital, Cantonment, Trichy

2HOD and Senior Consultant, Obstetrics and Gynaecology, Cantonment, Trichy

3Consultant Cardiologist and Electrophysiologist. Heart City, Trichy

Background

Atrial septal defect (ASD) is one of the commonest congenital heart diseases seen in pregnant mothers. They are classified into four types: ostium secundum (75% of cases), ostium primum (15-20% of cases), and sinus venosus (5-10% of cases) and, less commonly, an unroofed coronary sinus. It causes left to right shunting leading to right atrial and right ventricular enlargement and increased pulmonary blood flow. Women with coexisting severe pulmonary hypertension run a great risk of morbidity and mortality. Hemodynamic changes throughout gestation may increase the risk for complications, particularly in those with un-repaired ASDs. Arrhythmias are the most common cardiac event and occur in 4-5%, followed by paradoxical emboli in 2-5%. In this work, a case of uncorrected secundum with left to right shunt, complicated with severe pulmonary hypertension in a pregnant lady was reported.

Case presentation

A 29 years old female (gravida 4 para 2 live 2 and abortion 1) with last menstrual period on 9/3/2023 and expected date of delivery on 14/2/2023 had her regular checkups in outside hospital.

She was diagnosed to have hypothyroidism in her first month of conception. She had an iron sucrose injection of 3 doses at 5 months of gestation, presumably to address anemia.

Echo screening done, showed atrial septal defect with 36mm ostium secundum, moderate tricuspid regurgitation, severe pulmonary hypertension, ra/rv dilatation with left ventricular ejection fraction of 75%.

Hence, at the gestational age of 33 weeks and 4 days, she was referred to our hospital for cardiac disease complicating pregnancy for further management.

Menstrual history

Regular with 5/28 days cycle.

Marital history

Married since 10 years

Non consanguineous marriage

Past obstetric history

Her first two pregnancies were uneventful with full term LSCS, and both were healthy female children with the age of 9 years and 7 years.

Her third pregnancy ended in missed abortion at 3 months for which legal termination (MTP) was done.

Her fourth pregnancy was her present pregnancy.

Presenting Chief Complaints

She had complaints of lower abdominal pain for 3 hr from 8 pm on 29/10/2023 and complaints of difficulty in breathing since 9 pm on same day. She able to perceive fetal movements. No evidence of bleeding or leaking per vaginum.

On Examination

Patient conscious, oriented, Afebrile

Hemodynamically stable

Heart rate – 90/min

Blood pressure – 110/70 mm hg

Spo2 – 98% in room air

Per abdomen,

  • uterus 32 to 34 weeks
  • Mildly acting
  • Fetal heart sound present
  • Fetal movements good,
  • Cervix un-effaced with os closed.

Investigations

ECG

ECG-0

Echo Report

  1. Showed large ostium secundum. predominantly left to right shunt
  2. Right atrium and right ventricle dilated with d shaped septum
  3. Severe tricuspid regurgitation
  4. Severe pulmonary artery pressure with RVSP – 100 mm hg
  5. Mild tricuspid regurgitation
  6. Normal biventricular function.
ECG-1
ECG-2

Management

 

 

  1. After initial assessment in the emergency, patient was taken for emergency LSCS and bilateral tubal sterilization. Under antibiotic coverage, and under spinal anesthesia, she delivered a male baby of birth weight of 2.16 kg at 2.15 am on 30/10/2023.
  2. Post operatively patient was shifted with oxygen support to intensive care unit for further care.
  3. NT-pro BNP done was with in normal limits (321 pg/ml ) .
  4. Arterial blood gas analysis showed hypoxia.
  5. Patient was managed with tablet sildenafil 20 mg and diuretics were added, to reduce pulmonary artery pressures.
  6. On continuous monitoring in ICU with supportive medications and oxygen support.
  7. Her pulmonary pressures gradually improved and oxygen support was gradually tapered
  8. Shifted to ward with minimal oxygen support via nasal prongs. Oxygen support was gradually weaned off.
  9. Patient became comfortable in room air with the saturation of 88-90 % and was discharged in a stable condition.

Discussion

Atrial septal defect is the most prevalent type of congenital heart disease and one of the most commonly recognized congenital cardiac anomalies that present in adulthood.

The overall prevalence of ASD has been estimated to be 0.85 per 1,000 adults, which is likely an underestimate considering that some patients remain clinically asymptomatic, with a normal life expectancy.pregnancy in women with ASDs is generally well tolerated and considered to be at low risk for maternal and fetal morbidity and mortality.

This defect does not always require surgical or trans catheter closure. If the pregnant woman is asymptomatic (NYHA functional class I and II) without complications like heart failure, atrial arrhythmia or pulmonary hypertension or history of stroke, an expectant management during pregnancy is acceptable.

Pregnancy with an unrepaired ASD with complications appears to be associated with a higher risk of preeclampsia (7%), small for gestational age birth weight (21%) and fetal/perinatal mortality (2-3%).

Hemodynamic changes throughout gestation may increase the risk for complications both to the mother and fetus, particularly in those with uncorrected ASD and pulmonary hypertension.

In patients with unrepaired ASD, these pregnancy-induced physiological changes can aggravate right ventricular volume overload, with the potential to trigger heart failure and contribute to atrial dilation and the genesis of atrial arrhythmias (i.e., atrial fibrillation and flutter), this can result in a reduction in oxygenated blood in maternal and fetal circulations.

Conclusion

Pregnancy remains contraindicated in patients with asds associated with severe pulmonary hypertension due to poor maternal and fetal outcomes.prognosis of this type of patient is bad & ultimate solution is heart lung transplantaion. However, a multidisciplinary approach is important in managing pregnant mothers with large asd and pulmonary hypertension.

References

  1. Bredy C, Mongeon FP, Leduc L, Dore A, Khairy P. Pregnancy in adults with repaired/unrepaired atrial septal defect. J Thorac Dis. 2018 Sep;10(Suppl 24):S2945-S2952.
  2. Van der Bom T, Bouma BJ, Meijboom FJ, Zwinderman AH, Mulder BJ. The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation. Am Heart J. 2012 Oct;164(4):568-75.
  3. European Society of Gynecology (ESG); Association for European Paediatric Cardiology (AEPC); German Society for Gender Medicine (DGesGM); Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart JM. ESC Committee for Practice Guidelines. 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 Dec;32(24):3147-97.
  4. Canobbio MM, Warnes CA, Aboulhosn J, Connolly HM, Khanna A, Koos BJ, et al,. American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; Council on Functional Genomics and Translational Biology; and Council on Quality of Care and Outcomes Research. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation. 2017 Feb 21;135(8):e50-e87
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  6. Drenthen w, pieper pg, roos-hesselink jw, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J am coll cardiol 2007;49:2303-11. 10.1016/j.jacc.2007.03.027.
  7. Muhammad Zulkifli Konok, Mohd Khairi Othman, Zurkurnai Yusof, W. Yus Haniff W. Isa. Successful pregnancy in a patient with uncorrected atrial septal defect (ASD) complicated by pulmonary hypertension: A case report. 2023; 373(14).
  8. Konok, Muhammad & Othman, Mohd Khairi & Yusof, Zurkurnai & W Isa, W Y Haniff. (2023). Successful pregnancy in a patient with uncorrected atrial septal defect (ASD) complicated by pulmonary hypertension: A case report. International Journal of Cardiology. 373. 14.
Suchitra

Dr. N. Suchitra

HOD and Senior Consultant, Obstetrics and Gynaecology

Joseph

Dr. T. Joseph

Consultant Interventional Cardiologist

Ramya

Dr. Ramya

MEM Resident