ISSN : 2146-3123
E-ISSN : 2146-3131

Fatma Sevinç Şengül1, Pelin Ayyıldız1, Aysel Türkvatan2, Okan Yıldız3, Alper Güzeltaş1
1Clinic of Pediatric Cardiology, University of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey
2Clinic of Radiology, University of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey
3Clinic of Pediatric Cardiac Surgery, University of Health Sciences Turkey, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey
DOI : 10.4274/balkanmedj.galenos.2023.2023-2-65
Pages : 226-227

Case 1 was a 7.5-month-old male patient weighing 7.3 kg, who had been hospitalized three times due to bronchiolitis/pneumonia. On physical examination, a 3/6 systolic ejection murmur was heard at the left upper sternal border, and his oxygen saturation (SpO2) was 98%. Transthoracic echocardiography (TTE) revealed a patent foramen ovale with left-to-right shunt, moderate enlargement of the right ventricle, giant coronary sinus (CS) (Figure 1a, Video 1), and a partial anomalous pulmonary venous return (APVR) in which three pulmonary veins drained into the CS (Video 2). Computed tomography (CT) showed three pulmonary veins draining into the coronary sinus (Figure 1b,Video 3). This patient was referred to us with the diagnosis of total APVR.

Case 2 was a 9-month-old male patient weighing 8.5 kg. Similar to the first case, a 3/6 systolic ejection murmur was heard on physical examination. The patient’s SpO2 was 97%. TTE showed a small secundum atrial septal defect with a left-to-right shunt, enlargement of the right heart chamber, and drainage of three pulmonary veins into the CS. The diagnosis was confirmed by CT. The 3D PDF file of Case 2 demonstrates the modeling of three pulmonary veins that are drained into the CS (Appendix 1). This document is included in the 3D PDF HEART ATLAS project of the Turkish Society of Pediatric Cardiology and Cardiac Surgery. Both patients underwent surgery by unroofing the CS, and tolerated the process well. Postoperative echocardiography showed unobstructed pulmonary venous drainage into the left atrium.

The pulmonary venous connection to the coronary sinus occurs in approximately 3% of partial APVR.1 A careful echocardiographic evaluation is essential, and all pulmonary veins should be seen draining into the left atrium on TTE. Otherwise, partial APVR should be suspected. Additional examinations, such as Cardiac CT and magnetic resonance, and modeling of cardiac structures in 3D PDF can be performed to confirm the diagnosis.1-3 The most important feature distinguishing partial APVR from total APVR is that the interatrial shunt is left-to-right in the partial APVR and right-to-left in the total APVR as observed in TTE.

Acknowledgments: The authors would like to thank Kula et al. for their technical support on 3D PDF modeling. The 3D PDF file of Case 2 mentioned in this study has been provided as an attachment (Appendix 1). This document requires Adobe Reader to view it. As this 3D PDF technique is different from the standard techniques, watching the information video regarding the use of the 3D PDF file is suggested in Kula et al.’s3 study.

Informed Consent: Informed consent was obtained from the patient’s parents.

Authorship Contributions: Concept- F.S.Ş.; Design- F.S.Ş.;  Data Collection or Processing- O.Y.; Material- A.T.; Analysis or Interpretation- F.S.Ş.; Literature Search- F.S.Ş.;  Writing- F.S.Ş.; Critical Review- A.G.

Conflict of Interest: No conflict of interest was declared by the authors.

Video 1.

Transthoracic echocardiographic four-chamber view shows the dilated right heart chambers and coronary sinus of Case 1.

10.4274/balkanmedj.galenos.2023.2023-2-65.video1

Video 2.

Echocardiographic subcostal sagittal view shows the PFO with left-to-right shunt and pulmonary veins draining into the coronary sinus of Case 1.

10.4274/balkanmedj.galenos.2023.2023-2-65.video2

Video 3.

3D-computed tomography imaging shows the dilated coronary sinus, and three pulmonary veins drained into the coronary sinus of Case 1.

10.4274/balkanmedj.galenos.2023.2023-2-65.video3

REFERENCES

  1. Rajagopal R, Pandey NN, Sinha M, Sharma S. Cardiac Type of Partial Anomalous Pulmonary Venous Connection. Radiol Cardiothorac Imaging. 2020;2:e190100.
  2. Namkoong M, Choi KB, Lee C. Unusual type of partial anomalous pulmonary venous connection: Three pulmonary veins connected to the coronary sinus. J Card Surg. 2017;1-2.
  3. Kula S, Tunç O, Kavgacı A, Incedere F. Demonstrating a rare anatomical variation of cardiovascular system by using a new technique. Anatol J Cardiol. 2020;24:244-246.

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