Keywords

Cor triloculare biventriculare, pregnancy, congenital heart disease

Maternal heart disease complicates at least 1% of pregnancies and is one of the most important cause of maternal death.1 In developing countries, rheumatic heart disease remain the major cause, while in developed countries congenital heart diseases (CHD) are more prevalent due to improved survival of children with CHDs. Hemodynamic burden of pregnancy can unmask previously asymptomatic heart disease. Women with pre-existing but yet undiagnosed heart diseases are likely to present primarily to obstetrician and not to cardiologist. The prevalence of heart disease in pregnant women and its potentially life-threatening consequences justify a careful cardiac history, family history and physical examination in all pregnant women. Recognizing heart disease during pregnancy is challenging as physiological changes during pregnancy can cause signs and symptoms mimicking cardiac disease, e.g., fatigue, shortness of breath, edema and systolic ejection murmur.

CASE REPORT

A 19-year-old G2P1L0 female, married for 3 years with 7-month period of amenorrhea reported in labor room of Government Medical College and Rajindra Hospital, Patiala on 18/4/2014 (vide CR No. 3325) with complaints of pain abdomen and slight bleeding per vaginum (BPV) for the last 12 hours with no proper antinatal care. Ultrasonography (USG) already done on 1/4/14 showed single live intrauterine fetus with 20-21 weeks of gestation with intrauterine growth restriction (IUGR) and oligohydramnios, amniotic fluid index (AFI) 0.6 cm, placenta posterior showing 13 × 6.0 cmheterogeneous mass suggestive of retroplacental hemorrhage. Patient did not consult any doctor after this USG. Her first pregnancy was home conducted preterm delivery at 7½ months of gestation and baby died after 22 days (1 year back).

On admission, her vitals were: pulse rate 99/min, blood pressure (BP) 100/60 mmHg, respiratory rate 20/min, afebrile, chest was clear on auscultation. On per abdomen height of uterus was 28 weeks, abdomen was tense and uterine contractions were present. Fetal heart sound could not be localized. On per vaginum examination, cervix was 3 cm dilated, fully effaced. Her routine investigations were within normal limits. She delivered a dead male baby (macerated) with birth weight of 500 g after 3 hours of admission.

After about 1 hour of delivery patient had palpitations. On examination, uterus was well-retracted and BPV was within normal limits. Vitals at that time were pulse rate 130/min irregularly irregular, BP 110/70 mmHg;tachypnea was present. Her O2 saturation was 85%, cyanosis and clubbing were present. But no pallor, icterus, enema or lymphadenopathy (LAP) was present. Jugular venous pressure (JVP) was normal. On auscultation chest was clear with bilateral air entry equal. On cardiovascular examination, apex beat was present in left 5th intercostal space in midclavicular line, parasternal heave present, P2 palpable, S1 loud, ejection systolic murmur was present in pulmonary area. Central nervous system examination was normal. Provisional diagnosis of atrial fibrillation with cyanotic heart disease was made.

Patient was shifted to intensive coronary care unit (ICCU) and put on treatment in form of O2 inhalation, injection furosemide, tablet metoprolol 25 mg along with antibiotics. Her fluids intake was restricted and she was put on cardiac monitoring. Electrocardiography (ECG), chest radiography and echocardiogram was done. Chest X-ray was normal. ECG depicted RSR’ pattern in lead V1 suggestive of right bundle branch block (RBBB). Echocardiogram revealed congenital cyanotic heart disease with single common atrium (intra-atrial septum deficient), large perimembranous VSD (ventricular septal defect 22 mm), moderate pulmonary arterial hypertension (PAH), moderate mitral regurgitation and moderate tricuspid regurgitation. She was managed conservatively and responded well to the treatment. Patient was discharged on 7th postpartum day with stable vitals and advised to continue tablet metoprolol 25 mg b.i.d. and regular follow-up in Cardiology Department.

DISCUSSION

Cor triloculare biventriculare (single common atrium) is a very rare congenital disorder in adults with very few reports among adults.2 It is similar to a very large septal defect. This defect occurs as a result of lack of septum primum and septum secundum formation.

Clinical presentation includes mild tachypnea, cyanosis and clubbing of fingers and toes. Our patient was incidentally diagnosed because of the thrill discovered on routine physical examination. The patient presented here was essentially asymptomatic until the second stage of labor. Hemodynamic changes of labor had altered the haemodynamic balance with resultant decompensation.

Major hemodynamic changes occur during late pregnancy, labor, delivery and postpartum period. In patients with pre-existing cardiovascular disease (CVD), cardiac decompensation often coincides with this peak. Few cases have been reported among the whites, since the early twentieth century. The few reported cases of cor triloculare biventriculare include Abott’s in 1936, who reported five cases in her analysis of 1,000 CHD cases in United State.3 Brown et al also reported isolated case series with few surviving beyond middle age.4

Cunningham in 1948, reported a case of a man with cor triloculare biventriculare, pulmonary dilatation and membranous VSD who lived till 51 years of age.5 Ellis et al, Dubost and Blondaeu described similar patients who were between ages 3 and 31 years.6 Ellis-van Creveld syndrome is a rare combination of cor triloculare biventriculare, chordra and ectodermal dysplasia, polydactyly and most patients die in infancy.7 Baron also reported another case from Spain in 1962.8

Peachey and Robertson reported a case of cor triloculare biventriculare with hypoplasia of the tricuspid valve surviving to the age of 65 years in United Kingdom.9 Sangam et al recently reported a case of a stillborn male fetus of 35 weeks gestation with cor triloculare biventriculare and persistent left superior vena cava in India.10 Akintunde et al reported a similar case of cor triloculare biventriculare and pulmonary stenosis in Nigerian primigravid woman.11

Contraindications of pregnancy in cardiac diseases are:

Severe hypertension of any etiology

Severe fixed obstructive cardiac lesion

New York Heart Association (NYHA) Class III and IV heart failure

Left ventricular ejection fraction <40%

Prior peripartum cardiomyopathy (PPCM)

Dilated unstable aorta of 40-45 mm or above or severe cyanosis.

Cardiac diseases in pregnancy score (CARPREG) risk score has been shown to predict the adverse cardiac complications during pregnancy (Table 1):

It is composed of four clinical features

Each risk factor is associated with 1 point

Maternal cardiac event rate associated with 0, 1, >1 points is 5%, 27% and 75%, respectively.

Maternal mortality in women with this syndrome ranges from 30% to 50% with a 50% risk to fetus if mother survives. Mortality is frequently caused by thromboembolic events. Fetal risk due to maternal hypoxemia is substantial with a high incidence of fetal loss, premature delivery, IUGR and perinatal death. Because of considerable risk to fetus and mother, pregnancy is contraindicated. If pregnancy occurs therapeutic abortion is recommmended. Studies show that a low maternal oxygen saturation (<85%) is associated with a very low rate of live born infants (12%).

Women who choose to continue pregnancy are advised to restrict physical activity, use continuous oxygen at least for third trimester and consider use of pulmonary vasodilating drugs such as iloprost and prostacyclin. Anticoagulation is recommended during the third trimester and for 4 weeks after delivery.

The most vulnerable period for mother is labor, delivery and first week postpartum. Vaginal delivery, facilitated by vacuum or low forceps extraction is the method of choice. Cesarean delivery is associated with substantially higher mortality than the vaginal route. Anesthetic management includes central venous and arterial pressure monitoring, with maintenance of adequate systemic vascular resistance and intravenous volume and prevention of sudden increasing pulmonary vascular resistance.

REFERENCES

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3. Abbott ME. Atlas of congenital cardiac diseases. American Heart Association, New York, 1936.
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6. Ellis FH Jr, Kirklin JW, Swan HJ, Dushane JW, Edwards JE. Diagnosis and surgical treatment of common atrium (cor triloculare-biventriculare). Surgery 1959;45(1):160-72.
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11. Akintunde AA, Aremu AA, Akinboro AO, Akinlade MA. Cor triloculare biventriculare and pulmonary stenosis in a Nigerian primigravid woman: a case report. Int J Cardiovasc Res 2013;2:3.