Postpartum ischemic stroke due to persistent left superior vena cava to left atrium after DORV repair: a case report and literature review

Abstract

Background

Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly and is often associated with complex congenital heart disease (CHD). Venous drainage typically occurs into the right atrium via the coronary sinus (CS). However, in 10–20% of cases, it drains into the left atrium, producing a right-to-left shunt that may result in symptoms and cerebrovascular complications.

Case presentation

A 23-year-old woman with a history of double-outlet right ventricle corrected at age 13 presented in the postpartum period with dysarthria, left central facial palsy and left hemiparesis. Brain MRI angiography revealed right middle cerebral artery (M2) occlusion, and she underwent thrombolysis and thrombectomy. Suspecting a cardioembolic event, transesophageal echocardiography with left-arm agitated saline injection revealed early left atrial opacification and delayed right-sided filling via a residual ventricular septal defect —suggesting a PLSVC draining into the left atrium. Cardiac CT confirmed CS agenesis and a PLSVC connected to the left atrial roof. She was discharged on enoxaparin.

Conclusions

PLSVC is a vascular anomaly with an estimated prevalence of 0.2-3% in the general population and 1-12% among CHD patients. A dilated CS on transthoracic echocardiography should prompt a bubble test; early opacification of the left chambers after left-arm saline injection suggests drainage to the left atrium. Cardiac CT, MRI or invasive angiography can clarify the anatomy.

PLSVC has clinical implications including technical considerations for percutaneous procedures and cardiac surgery, a predisposition for arrhythmias, and —when draining into the left atrium— an increased risk of cerebrovascular events. Closure of a PLSVC draining into the left atrium should be considered in symptomatic patients or those at high risk of paradoxical embolism, following anatomical assessment.

In pregnant women with CHD, cardiac risk assessment, multimodal imaging and multidisciplinary follow-up are crucial due to associated hemodynamic changes and hypercoagulable state.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12872-025-04922-2.

Background

Adult congenital heart disease refers to the persistence of structural anomalies of the heart and/or great vessels present at birth into adulthood, posing a growing burden on healthcare systems. Over the past four decades, advances in surgery and interventional cardiology have reduced infant mortality from congenital heart disease (CHD) nearly threefold, allowing over 85% of affected individuals to reach adulthood. With an incidence of approximately 8 per 1,000 live births, currently an estimated 50 million people worldwide live with CHD [1].

Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly and is often associated with complex CHD. Venous drainage typically occurs into the right atrium (RA) via the coronary sinus (CS). However, in some cases, it drains directly into the left atrium (LA), creating a right-to-left shunt, which has clinical significance due to its potential to cause symptoms and cerebrovascular complications [2, 3].

Case presentation

A 23-year-old female patient, gravida 1, para 0 (G1P0), at 36 weeks of gestation with a history of double outlet right ventricle (DORV) corrected at age 13, was transferred to a tertiary hospital due to suspicion of placenta previa, intrauterine growth restriction and maternal CHD without specialized adult CHD follow-up.

On admission she was normotensive, without respiratory distress. Physical exam revealed a gravid uterus with a single live fetus, a fetal heart rate of 138 beats per minute and no uterine activity. A transabdominal obstetric ultrasound showed a normal biophysical profile, intrauterine growth restriction, total placenta previa with a highly vascular component, a large placental volume and a high risk of placenta accreta. Given these findings, a cesarean section was performed following the placenta accreta protocol. Intraoperative findings revealed an abdominal ectopic pregnancy attached to the intestine, appendix, bladder, abdominal wall, and uterine horn. A 2125-gram female newborn was delivered. Due to intraoperative bleeding, the patient required multiple red blood cell transfusions and intensive care unit monitoring. She and her newborn were discharged six days later.

One day after discharge, she was readmitted to the emergency department following a transient loss of consciousness two hours earlier, accompanied by dysarthria, left central facial palsy and left-sided hemiparesis. On admission, her blood pressure was 110/70 mmHg, heart rate 78 beats per minute, temperature 36°C and oxygen saturation 99%. The patient was alert and oriented, with severe dysarthria. She followed instructions and exhibited 3/5 strength in the left upper limb and 2/5 strength in the left lower limb, with preserved motor function on the right side. The National Institutes of Health Stroke Scale (NIHSS) score was 9. A holosystolic murmur was audible at all auscultation points, most prominently at the aortic area.

A noncontrast head computed tomography (CT) scan showed no intracranial hemorrhage. Magnetic resonance angiography confirmed an acute ischemic stroke in the right middle cerebral artery (M2 segment). Thrombolysis and thrombectomy were performed without complications. During cerebral angiography via the right femoral artery, multiple arterial thrombi were aspirated. An occlusion of the external iliac artery was identified and treated with catheter-directed thrombolysis. Anticoagulation with enoxaparin was initiated.

A transesophageal echocardiogram (TEE) was performed due to suspicion of a cardioembolic stroke in a young postpartum patient with a history of corrected congenital heart disease (CHD) and multiple arterial thrombi. The differential diagnosis included paradoxical embolism through an intracardiac shunt, thromboembolism related to arrhythmias such as atrial fibrillation or flutter, infective endocarditis, and thrombus formation on prosthetic material (e.g., the left ventricular outflow tract (LVOT) conduit or surgical patches), in the context of the hypercoagulable state associated with the postpartum period.

TEE revealed segmental wall motion abnormalities in the inferior left ventricular wall, with a preserved ejection fraction of 55–60%. Right ventricular size and systolic function were normal. The right atrium was severely dilated (indexed volume 52 mL/m²). A corrected DORV was noted, with mild obstruction of the LVOT prosthetic conduit (Fig. 1) and a residual ventricular septal defect (VSD) between the LVOT and the RA measuring 4 mm (17% of the LVOT diameter), resulting in a restrictive shunt (peak velocity 5.5 m/s, peak gradient 121 mmHg) (See supplementary file Video 1). Due to the absence of specialized adult CHD follow-up, no recent cardiac imaging had been performed, and the VSD had not been previously documented. Given the lack of hemodynamic impact, it was considered unlikely to represent a newly developed lesion.

Fig. 1.

Transthoracic echocardiogram. A Corrected double outlet right ventricle (DORV) with a prosthetic conduit connecting the left ventricular outflow tract to the aortic valve. B Color Doppler imaging showing mild obstruction of the left ventricular outflow tract with adequate aortic valve function

Moderate tricuspid regurgitation was present (biplane vena contracta diameter: 7 mm), functional in origin due to right atrial dilation. The estimated pulmonary artery systolic pressure (PASP) was 41 mmHg, suggesting intermediate probability of pulmonary hypertension, which did not account for the degree of tricuspid regurgitation.

Contrast injection via the left upper limb (Fig. 2, See supplementary file Video 2) demonstrated early opacification of the left heart chambers, with contrast reaching the atrioventricular junction before the right heart chambers, suggesting a PLSVC draining into the LA. Delayed contrast arrival in the right heart chambers was attributed to shunting from the VSD into the RA. Subsequent contrast injection via the right upper limb showed early right atrial filling with a negative contrast effect, consistent with left-to-right shunting across the VSD.

Fig. 2.

A, B Transthoracic echocardiogram. Agitated saline injection via the left upper limb resulted in rapid contrast filling (yellow arrows) of the left heart chambers—reaching the atrioventricular junction (A) and the left ventricle (B) before the right heart chambers. C Transesophageal echocardiogram (bicaval view). Agitated saline injection via the left upper limb leading to early dense contrast opacification of the left atrium and delayed faint opacification of the right atrium. D Transthoracic echocardiogram. Contrast injection via the right upper limb showing early right chambers filling

Cardiac CT angiography was performed for a detailed anatomical assessment. Using a multidetector scanner, volumetric acquisition of the heart was performed with prospective electrocardiographic gating during intravenous contrast administration. Curved multiplanar and volume-rendered angiographic reconstructions were generated to assess intracardiac anatomy and vascular connections. Dilation of the left and right heart chambers was observed. A subaortic fold measuring 2.0 × 1.5 cm in transverse and anteroposterior dimensions was noted, narrowing the LVOT (Fig. 3). Agenesis of the coronary sinus was identified, with the presence of a left superior vena cava connecting to the roof of the left atrium (Fig. 4).

Fig. 3.

Cardiac CT angiography. Distortion in the contours of the left ventricle outflow tract due to the presence of a subaortic fold that narrows the outflow tract (thick yellow arrow in A), which measures 2.0 × 1.5 cm transversely and anteroposteriorly (thin yellow arrows in B)

Fig. 4.

Cardiac CT angiography. A, B Persistent left superior vena cava (*) connecting to the roof of the left atrium. C, D Absence of coronary sinus (thin yellow arrows) with posterior interventricular vein draining directly to the right atrium (thick yellow arrows)

The patient had a stable neurological course, with persistent left hemiparesis and severe dysarthria. She exhibited 3/5 strength in the left upper and lower limbs, with Ashworth grade I spasticity on the left side. Use of venous access in the left upper limb was contraindicated. A multidisciplinary evaluation prior to discharge led to the decision to continue anticoagulation therapy with enoxaparin, refer the patient for outpatient hematology assessment to better estimate the risk of thromboembolic recurrence, and consider endovascular closure on an outpatient basis, given the elevated risk of complications shortly after cesarean delivery, thrombolysis, and thrombectomy. Physical medicine and rehabilitation recommended outpatient physical, occupational, and speech therapy.

Discussion

CHD is the most common pre-existing cardiovascular condition present during pregnancy in the western world, accounting for 75–82% of cases. The maternal risk of cardiac complications must be assessed, ideally in timely pre-pregnancy counselling. The modified World Health Organization classification (mWHO) is useful for the assessment of disease-specific maternal cardiac risk from risk class I to IV, allowing for planning of follow-up and interventions. Our patient is classified into group III because of a corrected complex CHD with residual lesions, presence of moderate tricuspid regurgitation and PLSVC draining to the LA. This risk class indicates a significantly increased risk of maternal-fetal morbidity and mortality, with a maternal cardiac event rate ranging from 19 to 27%. Pregnancy follow-up should be conducted in a specialized center [4].

DORV is a CHD defined as a heart with both arterial trunks supported in their greater part by a morphologically right ventricle, an anomaly always associated with a VSD, which serves as the only path for oxygenated blood to exit the left ventricle. The clinical presentation depends on the location of the VSD and the degree of pulmonary stenosis. It may manifest as heart failure due to pulmonary hyperflow or cyanosis if pulmonary stenosis is present. The surgical repair approach depends on the anatomy and associated defects. Patients with a history of DORV correction must be closely monitored during pregnancy, as related hemodynamic changes can exacerbate residual shunts or outflow tract obstructions, arrhythmias due to surgical scars or heart failure if ventricular function is impaired [5].

Conotruncal anomalies frequently coexist with other structural heart defects, including PLSVC, the most common congenital thoracic venous anomaly. PLSVC originates at the junction of the left subclavian vein and the internal jugular vein. It occurs in approximately 0.2 to 3% of the general population and is found in 1 to 12% of individuals with CHD. According to the study by Ari et al., the most frequent coexistent CHDs are VSD, DORV and tetralogy of Fallot [6].

In 80% to 92% of cases, PLSVC drains into the RA through a dilated CS. However, in 10% to 20% of cases, it drains into the LA due to an atrial septal defect (ASD), CS agenesis, or an unroofed CS, creating a right-to-left shunt. This scenario is usually associated with additional cardiac anomalies. The combination of an ASD and a PLSVC draining into the LA via an unroofed CS is known as Raghib syndrome. Another possible variant is CS ostial atresia, where the PLSVC serves as the primary drainage route for coronary veins in the absence of collateral pathways [7]. In 90% of cases, the right superior vena cava is present and may or may not be connected to the PLSVC via the left brachiocephalic (bridging) vein. In the remaining 10%, an isolated PLSVC is found, an anomaly often linked to cardiac situs disorders [8]. Our patient had a PLSVC draining to the LA and CS agenesis. The right superior vena cava was present, but the imaging modalities used did not allow assessment of a bridging vein.

Most patients with PLSVC in the absence of accompanying defects are asymptomatic. PLSVC draining to the RA often causes CS enlargement, which, if significant, may reduce the LA size leading to decreased cardiac output. Additionally, compression of the atrioventricular node and His bundle may result in atrial or ventricular arrhythmias. A study by Yung Gi Kim et al. demonstrated that PLSVC plays a significant role in triggering and sustaining atrial fibrillation [9]. Drainage to the LA may cause paradoxical embolism to systemic circulation, leading to complications such as stroke and brain abscesses. A significant right-to-left shunt could present with cyanosis, functional class reduction or syncope [7]. Our patient presented with a cardioembolic stroke in the postpartum period, related to the hypercoagulable state of puerperium and paradoxical embolism. She did not have symptoms related to hemodynamic impact of the shunt.

Technical considerations must be considered for patients with a PLSVC undergoing procedures requiring intravenous access to the right heart chambers and CS, including central venous catheter insertion and placement of cardiac resynchronization therapy leads, as doing so in undiagnosed patients can result in difficult insertion, incorrect positioning or serious complications such as arrhythmias and myocardial perforation [3]. In surgery, retrograde cardioplegia administration is relatively contraindicated. A CS catheter balloon may fail to fully occlude a dilated CS and cardioplegia partially flows to the left internal jugular and left subclavian veins, leading to inadequate retrograde delivery to the myocardium [2].

These clinical implications underscore the importance of a thorough characterization of thoracic venous anatomy in patients with a history of CHD, particularly during pregnancy —a condition that induces significant hemodynamic changes and a hypercoagulable state [4]. Interpretation of multimodal cardiac imaging requires expertise in CHD due to the complexity of native or postoperative anatomy. Transthoracic echocardiography is the most frequently performed cardiovascular image. PLSVC may be suspected upon visualization of a dilated CS followed by injection of agitated saline from bilateral peripheral veins, showing opacification of the CS or LA from the anomalous drainage of the left arm [10]. CT angiography allows for high-resolution three-dimensional visualization of the venous anatomy, characterizing the course and drainage of the PSLVC. Magnetic resonance imaging (MRI), particularly with venography sequences, offers similar anatomical detail without radiation exposure and is excellent for the assessment of associated CHD. Phase-contrast MRI is useful for hemodynamic assessment, especially in cases of drainage to the LA [2, 10]. Lastly, catheter angiography is the gold standard for confirming drainage patterns if noninvasive imaging is inconclusive and may be used when planning electrophysiology procedures [7].

PLSVC draining into the CS without hemodynamic compromise requires no intervention. However, if the PLSVC drains into the LA, creating a right-to-left shunt, treatment may be necessary to prevent systemic desaturation and paradoxical embolism [2, 7]. When considering intervention, a thorough assessment of alternative venous return pathways is essential. If a left brachiocephalic (bridging) vein is present, surgical or percutaneous closure of the PLSVC can be considered, as blood flow is redirected to the right superior vena cava. If the bridging vein is absent, venous grafting of a bridging vein or surgical reimplantation of the PLSVC into the RA is required to ensure adequate venous return from the left arm and the left side of the head and neck [11]. In this case, the selected approach included anticoagulation with low-molecular-weight heparin, ambulatory rehabilitation and follow-up by cardiology and hematology. Endovascular closure was deferred to an outpatient setting due to the high procedural risk associated with recent cesarean delivery, thrombolysis, and thrombectomy.

Conclusions

The prevalence of CHD in adults is increasing due to improved life expectancy, highlighting the need for specialized adult CHD centers. In pregnant women with a history of CHD, cardiac risk assessment, detailed anatomical evaluation using multimodal cardiac imaging, and multidisciplinary follow-up are essential, given the associated hemodynamic changes and hypercoagulable state.

A venous anomaly often associated with complex CHD is PLSVC, which should be actively investigated due to its clinical implications, including technical considerations in surgical and percutaneous procedures, a predisposition to atrial and ventricular arrhythmias, and —in cases where the PLSVC drains into the left atrium— an increased risk of cerebrovascular events due to paradoxical embolism.

Abbreviations

ASD

Atrial septal defect

CHD

Congenital heart disease

CS

Coronary sinus

CT

Computed tomography

DORV

Double-outlet right ventricle

G1P0

Gravida 1, Para 0

LA

Left atrium

LVOT

Left ventricular outflow tract

MRI

Magnetic resonance imaging

mWHO

Modified World Health Organization classification

NIHSS

National Institutes of Health Stroke Scale

PLSVC

Persistent left superior vena cava

PASP

Pulmonary artery systolic pressure

RA

Right atrium

TEE

Transesophageal echocardiogram

VSD

Ventricular septal defect

Authors’ contributions

P.A.C., J.A.Z. and M.J.R. conceptualized the case report, reviewed the literature and drafted the manuscript. J.S. and P.O. selected the diagnostic imaging figures, interpreted the imaging findings and contributed insights with their expertise on the field. C.E.V. and C.A.C. led the clinical management of the patient, reviewed the manuscript and provided critical input on the clinical aspects of the case.

Funding

This study was not supported by any external funding.

Clinical trial number: not applicable.

Data availability

No datasets were generated or analysed during the current study.

Declarations

Ethics approval and consent to participate

This case report was reviewed and approved by the Institutional Review Board (IRB) of Fundación Valle del Lili, Cali, Colombia. The patient provided informed consent for the use and publication of their medical information, and all identifying details have been anonymized to ensure confidentiality.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare no competing interests.