Heart failure in a patient with Glenn circulation during secondary pregnancy with a single ventricle: A rare case report

Single ventricle refers to cardiac malformations characterized by severe ventricular septal hypoplasia or absence. Clinical manifestations include cyanosis, growth retardation, and symptoms of congestive heart failure. ¹ The Fontan procedure was the standard surgery. ² It separates systemic and pulmonary circulation, with over 85% of patients surviving beyond 30 years. ³ The Glenn procedure, as an intermediate palliative surgery, is associated with lower survival rates. ⁴

Women with Fontan circulation may safely undergo pregnancy supervision and delivery. Conversely, reports on pregnancies without Fontan circulation are scarce.

A 36‐year‐old woman was admitted to our hospital at 32 weeks of gestation due to “chest tightness and dyspnea for 10 days.” Notably, the patient had been diagnosed with “double outlet right ventricle (DORV)” in childhood. Due to financial constraints, she didn't undergo palliative Glenn surgery until 22 and failed to undergo further definitive Fontan surgery. One year later, she conceived and naturally delivered a male infant at 32 weeks without regular prenatal check‐ups but reported no discomfort.

In this instance, she did not heed medical advice to terminate the current pregnancy. Throughout her pregnancy, she had only a few irregular check‐ups. Before 30 weeks of gestation, no specific discomfort was reported. Ten days prior, she experienced chest tightness and dyspnea. Three days later, the patient presented to a primary care facility, receiving fetal heart rate monitoring, oxygen therapy, and antenatal corticosteroid therapy. However, her symptoms continued to worsen, requiring an orthopneic position to sleep at night. Upon referral to our hospital, cardiac ultrasound revealed “dextrocardia, single atrium, single ventricle, aortic valve stenosis, enlarged pulmonary artery, and mild to moderate atrioventricular valve regurgitation” (Figure 1a). Her vital signs included a pulse of 126 beats/min, respiratory rate of 18 breaths/min, blood pressure of 97/61 mmHg, and oxygen saturation of 87%. Physical examination showed jugular venous distension, slightly coarse breath sounds bilaterally without dry or moist rales, clubbing of fingers, and varicose veins without significant edema in the lower extremities (Figure 1b).

FIGURE 1.

Abnormal cardiac structure and physical examination findings in the patient. (a) Apical view and color Doppler echocardiography images and post‐Glenn procedure cardiac circulation diagram. (b) Physical examination findings of the patient included jugular venous distension, clubbing of fingers and varicose veins in the lower extremities.

After admission, a multidisciplinary consultation was carried out. Given the patient's cardiac malformation, accompanied by evident signs of acute heart failure like hypoxemia and tachycardia, and that fetal lung maturation therapy had already been completed, it was ultimately decided to perform a surgery to terminate, followed by anti‐heart failure treatment. Consequently, an elective cesarean section was performed under general anesthesia, with a female infant weighing 1380 g. The Apgar scores were 3/10 at 1 min, and 9/10 at 5 min. The infant was transferred to the neonatal intensive care unit (NICU) for respiratory support. After 2 weeks, the newborn was reunited with the mother.

Postoperatively, the patient's blood pressure and heart rate gradually returned to normal, and finger oxygen saturation remained low. Five days postoperatively, the patient experienced esophageal bleeding. Following treatment for hemostasis, the patient improved and was discharged on postoperative day 18.

In patients with a single ventricle, systemic and pulmonary blood mix within the cardiac chamber. This leads to inadequately oxygenated blood being delivered via the aorta, resulting in congenital cyanosis. In the Glenn shunt, the ductus arteriosus is ligated, the inferior vena cava is interrupted, and the superior vena cava is anastomosed end‐to‐side with the ipsilateral pulmonary artery. ¹

The patient remained in a state of elevated central venous pressure and low cardiac output after Glenn. During the second pregnancy, the reduction in vascular resistance and increased blood volume associated with gestation could not be met by the output of a functional single ventricle, ultimately leading to acute heart failure.

Given that patients with Glenn circulation are at a higher risk of heart failure, stricter indications for pregnancy should be established than those post‐Fontan. A 23‐year‐old multipara post‐Glenn who underwent surgical delivery at 38 weeks was reported as having no heart failure symptoms. ⁵ However, a 26‐year‐old primigravida with Glenn circulation developed acute heart failure at 36 weeks and underwent cesarean section after 48 h of anti‐heart failure treatment. ⁶

Due to a lack of large‐sample data, our study can only suggest that increasing age and time since palliative surgery are associated with poor outcomes. Early signs of heart failure should be detected during pregnancy. Thirty weeks appears to be a high‐risk period for heart failure, and prenatal examinations should include appropriate cardiac‐related assessments. If heart failure can be corrected, efforts should be made to prolong the gestational age to improve neonatal prognosis.

AUTHOR CONTRIBUTIONS

Lu Jiang drafted the initial manuscript, Xinyuan Teng collected clinical data, and Liquan Wang revised the manuscript and provided additional knowledge content. All authors approved the final submission.

FUNDING INFORMATION

This work was financially supported by Zhejiang Provincial Natural Science Foundation of China (LMS25H040003).

CONFLICT OF INTEREST STATEMENT

None declared.

ETHICS STATEMENT

Informed consent was obtained, and the study was approved by the Ethics Committee of the Second Affiliated Hospital of Zhejiang University School of Medicine (approval no.: LunShenYan 2025, no. 0397).

DATA AVAILABILITY STATEMENT

Data sharing is not applicable to this article as no new data were created or analyzed in this study.