Sinus Venous Atrial Septal Defect (SVASD)

Nandhini1*, Nagajothi2, Umarani3, Jayamenon4

1Staff Nurse, Kauvery Hospital, Heart city, Trichy, Tamil Nadu

2OPD Incharge, Kauvery Hospital, Heart city, Trichy, Tamil Nadu

3Assistant Nursing Superintendent, Kauvery Hospital, Heart city, Trichy, Tamil Nadu

4Nursing Superintendent, Kauvery Hospital, Heart city, Trichy, Tamil Nadu

*Correspondence

Abstract

Sinus venosus atrial septal defect (SVASD) is an uncommon congenital cardiac anomaly accounting for 5–10% of atrial septal defects. It is frequently associated with partial anomalous venous return (PAPVR), resulting in significant left-to-right shunting, right heart volume overload and progressive pulmonary vascular changes if left untreated. Early diagnosis using multimodality imaging is essential for timely surgical correction.

Key words: Sinus venosus atrial septal defect (SVASD); Partial anomalous venous return (PAPVR)

Introduction

SV-ASD is a rare subtype of ASD, comprising 5–10% of all cases. Unlike secundum defects, it occurs at the SVC (or) IVC-right atrial junction due to incomplete sinus venous septation. SVC-type defects are most common & are frequently associated with PAPVR.

This defect causes a left-right shunt, leading to right heart volume overload & potential pulmonary HTN if uncorrected. SVD is often missed on routine TTE & typically requires TEE, CT or MRI for diagnosis because of its location & associated anomalous veins. Surgical repair is standard treatment, as device closure is usually not possible.

 

Case Presentation

A 25 yrs old female with a chief complaint of breathlessness for 1 month duration. She was evaluated for CHD. ECG demonstrated sinus rhythm with a borderline short PR interval & features suggestive of right heart enlargement. TTE revealed preserved LV function (EF–60%) & findings suggestive of sinus venous ASD. Cardiac CT-angiogram confirmed an 8 mm SVASD located near SVC to right atrial junction. The RA, RV dilated, indicated chronic right side volume overload. The study also demonstrated partial anomalous pulmonary venous return, with the right upper pulmonary vein draining into SVC.

In addition, a persistent left SVC draining into right atrium via coronary sinus was identified. The main pulmonary artery was dilated to 3.9 cm, while both lower pulmonary veins drain normally into Left atrium.

Routine hematological, renal & coagulation parameters were within acceptable limits for intervention.

History of presenting illness

Patient presented to OPD with complaints of breathlessness for 1 month. Initially evaluated at a local hospital & referred for further management. During OPD evaluation, ECHO was advised along with CT-angio. TEE & CT-angio were performed on subsequent dates. No history of chest pain, dyspnea at rest, palpitations, swelling, abdominal pain, or vomiting was reported.

Clinical Findings

Patient was conscious, oriented & co-operative. Vitals were stable.

  • HR: 86 bpm
  • BP: 110/70 mmHg
  • RR: 20/min
  • SpO₂: 98% on room air
  • S1 S2: Present
  • CNS: Alert

Lab Investigations

  • Hemogram, RFT, LFT, and electrolytes are largely within normal limits. Mildly deranged lipid profile.
  • Total cholesterol: 200 mg/dL
  • LDL: 196.8 mg/dL

Other Investigations

Cardiac CT

Chest X-ray

ECHO Impression

  • EF – 60%
  • Dilated right atrium & RV
  • Dilated pulmonary artery
  • Severe TR
  • Suspected Pulmonary HTN
  • SVASD noted
  • CT-Angio: Sinus venosus ASD & anomalous pulmonary venous drainage & persistent left SVC. Cardiomegaly & right heart chamber dilatation.

Lab Investigations

  • Hemogram, RFT, LFT, and electrolytes are largely within normal limits. Mildly deranged lipid profile.
  • Total cholesterol: 200 mg/dL
  • LDL: 196.8 mg/dL

Diagnosis

  • SVASD with Left → Right shunt, anomalous pulmonary venous return, and suspected pulmonary arterial HTN.
  • ACHD
  • Sinus venous ASD
  • Normal LV

Management

Medical Management

  • Started on oral PAH therapy 20 mg OD.
  • IV fluids DNS 60 mL/hr, heparin infusion, Meropenem 500 mg IV.
  • Underwent device closure procedure for sinus venous ASD.
  • Procedure: Zephyr Shunt 18 × 79 mm with no residual leak.
  • Ecospirin 75 mg PO OD
  • Clopilet 75 mg PO OD
  • Dolo 650 mg TDS PO
  • Para IV SOS
  • Patient intubated and ventilated in PRVC mode during the procedure. Extubated post-procedure.
  • Hemodynamically stable post-procedure.
  • SpO₂ maintained – 98%.

Nursing Aspects: Patient independent in all ADLs. DVT risk score 0 – Low risk. Pressure sore risk score 14 – Low risk per institutional scale. Fall precautions initiated

Nursing Management: Pre- and post-procedure monitoring, vital signs, I/O charting, ventilator care and medication administration.

Discharge advice

Drug nameDoseFrequency
T. Ecospirin75 mgOD
T.Clopilet75 mgOD
T. PAH20 mgBD
T. Dolo650 mgSOS
T. Apixaban5 mgBD
T. Pantocid40 mgOD
Syp. Alex10 mlSOS

Patient Education

Counselled on PAH medication adherence, wound/device site care, activity progression, & symptoms to report. Psychological support is provided for a young adult with a new cardiac diagnosis. Nursing vigilance and post-device closure contributed to early mobilization & stable recovery.

Outcome

Post device closure of sinus venous ASD, the patient remained hemodynamically stable. She was weaned from mechanical ventilation & extubated successfully after the procedure. Oxygen saturation was maintained at 98% on room air. No immediate post-procedural complications such as residual shunt, device embolization, arrhythmia or bleeding were noted. Right heart strain improved on clinical assessment.

Conclusion

The patient was discharged in stable and ambulatory condition after successful device closure of sinus venous ASD. A 25-year-old female, was a known case of CHD with SV-ASD with left → right shunt & normal biventricular function, was admitted for SV ASD. The patient underwent successful sinus venosus ASD stenting. Her procedure and post-procedure period were well & stable. The patient was treated with all possible supportive measures. Normal diet advised.

References

  1. Otto, C. M., C. M., & Bonow, R. O. (2021). Braunwald’s heart disease: A textbook of cardiovascular medicine (12th ed.). Elsevier.
  2. Kliegman, R. M., St. Geme, J. W., Blum, N. J., Shah, S. S., Tasker, R. C., & Wilson, K. M. (2023). Nelson textbook of pediatrics (22nd ed.). Elsevier.
  3. Baumgartner, H., De Backer, J., Babu-Narayan, S. V., et al. (2021). 2020 ESC Guidelines for the management of adult congenital heart disease. European Heart Journal, 42(6), 563–645.
  4. Warnes, C. A., Williams, R. G., Bashore, T. M., et al. (2008). ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. Journal of the American College of Cardiology, 52(23), e143–e263.

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