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Case report
Haruhiko Sugimori
New Tokyo Hospital
Corresponding Author
ORCiD: https://orcid.org/0000-0002-9920-119X
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background: Isolated coronary sinus atrial septal defect (ASD) is defined as a coronary sinus unroofed in the terminal portion without a persistent left superior vena cava and other anomalies. This defect is rare and part of a wide spectrum of unroofed coronary sinus syndromes. Recently, several reports have described this finding. We searched the hospital’s database to determine the incidence of this defect, and to raise awareness of this condition, we discussed the findings from five patients with coronary sinus ASD who underwent surgical repair.
Case presentation: The patients were three women and two men with an age range of 63–77 years. All patients underwent transthoracic echocardiography and computed tomography, and one underwent magnetic resonance imaging. In two patients, the defect was found unexpectedly intraoperatively; left-to-right shunting was apparent in the other three patients preoperatively. The pulmonary-to-systemic blood flow ratio ranged from 1.42 to 3.1 following cardiac catheterization, and oxygen saturation step-up was seen on the right side of the heart. Valvular regurgitation was seen in 4/5 patients with the mitral, tricuspid, and aortic valves involved in different combinations and to different degrees. Right atrial and ventricular dilation were seen in 4/5 patients; three patients had left atrial dilation. Three patients experienced atrial fibrillation, and one of these also experienced paroxysmal ventricular contractions. All patients underwent surgical repair, and some underwent multiple procedures. One patient who had previously undergone kidney transplantation died approximately 1 year postoperatively; the remaining four patients are currently experiencing good activities of daily living without symptoms.
Conclusions: Coronary sinus ASD (Kirklin and Barratt–Boyes type IV unroofed coronary sinus syndrome) comprised 1.3% of adult congenital heart surgeries and 0.07% of adult open-heart surgeries in our hospital from 1999 to 2019. Our hospital performs cardiac surgery mainly for patients with acquired cardiac disease, and coronary sinus ASD is rare. Early diagnosis and, in symptomatic patients (especially those with blood access shunts, which may overload the heart), early surgical repair are important. The poorly prognostic case in our series is noteworthy, as similar cases have not been reported previously.
Keywords
Case series , Coronary sinus atrial septal defect , Unroofed coronary sinus syndrome , Poor prognosis , Renal dialysis , Adult congenital heart disease
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This is a list of supplementary files associated with this preprint. Click to download.
- CSASDsuppletable1.pdf
Supplemental Table 1 Number of congenital heart disease and open-heart surgeries performed in the past 20 years The numbers in parentheses represent the number of concomitant anomalies, as described precisely below. We identified one case of VSD (type I) with concurrent RVOT stenosis in 2002; one case of secundum ASD with PLSVC in 2003; one case of secundum ASD with sinus venosus ASD and one case of secundum ASD with PDA in 2005; one case of BAV with PDA in 2006; one case of secundum ASD with VSD (type II) and one case of sinus venosus ASD with PAPVR in 2007; one case of sinus venosus ASD with PAPVR and PLSVC in 2008; one case of BAV with PDA in 2009; one case of PDA with CoA in 2010; one case of secundum ASD with PA stenosis in 2012; one case of secundum ASD with PA aneurysm in 2013; one case of BAV with CPAF in 2014; one case of VSD (type II) with a double-chamber RV in 2015; and one case of sinus venosus ASD with concomitant PAPVR in 2017. The details of “others” in Table 6 are as follows: one case of QAV in 1999 and one in 2016, two cases of QAV in 2005, one case of aorto-pulmonary arterial fistula in 2008, and one case of PA aneurysm in 2013. In the past 20 years, the redo operations were as follows. Secundum ASD patch reclosure was performed because of detachment of a previous ASD patch in a 62-yearold woman in 2005, an 81-year-old woman in 2012, and a 45-year-old man in 2015. MVP and TAP were performed because of recurrent MR and TR after atrioventricular septal defect repair was performed in a 68year-old woman in 2008. Mitral valve replacement and MVP were performed because of recurrent MR after primary ASD repair in a 73-year-old man in 2009 and a 70-year-old woman in 2013. PDA division and closure because of recurrent PDA was performed in a 73-year-old woman in 2010. ASD atrial septal defect, URCS unroofed coronary sinus, VSD ventricular septal defect, I type I VSD, II type II VSD, PAPVR partial anomalous pulmonary venous return, AVSD atrioventricular septal defect, ToF tetralogy of Fallot, BAV bicuspid aortic valve, CoA coarctation of the aorta, PDA patent ductus arteriosus, Ebstein Ebstein anomaly, CPAF coronary artery–pulmonary artery fistula, OHS open-heart surgery, PA pulmonary artery, QAV quadricuspid aortic valve
- theCAREchecklist.pdf
- CSASDTablesJCS.pdf
Table 1 Background characteristics of patients with coronary sinus atrial septal defect BSA body surface area, NSR normal sinus rhythm, AF atrial fibrillation, HT hypertension, DM diabetes mellitus, DL dyslipidemia, OMI old myocardial infarction, COPD chronic obstructive pulmonary disease, CKD chronic kidney disease, HD hemodialysis, PCI percutaneous coronary intervention, RFCA radiofrequency catheter ablation, cesarean sec. cesarean section Table 2 Preoperative laboratory data WBC white blood cells, RBC red blood cells, Hb hemoglobin, Plt platelets, TP total protein, AST aspartate transaminase, ALT alanine transaminase, BUN blood urea nitrogen, Cre creatinine, eGFR estimated glomerular filtration rate, HbA1c hemoglobin A1C, BNP brain natriuretic peptide Table 3 Preoperative transthoracic echocardiography data BSA body surface area, CS coronary sinus, Qp/Qs pulmonary-to-systemic blood flow ratio, RA 4ch right atrium diameter on four-chamber view, RV 4ch right ventricular diameter on four-chamber view, TR tricuspid regurgitation, TRPG tricuspid regurgitant pressure gradient, LVIVST left ventricular interventricular septal thickness, PWT LV posterior wall thickness, Dd LV end-diastolic dimension, Ds LV end-systolic dimension, EF LV ejection fraction, MR mitral regurgitation, AR aortic regurgitation, PR pulmonary regurgitation Table 4 Cardiac catheter flow study and pressure study results Qp/Qs pulmonary-to-systemic blood flow ratio, L to R left to right, R to L right to left, PAP s/m pulmonary artery pressure systolic/mean, PCWP mean pulmonary capillary wedge pressure, C.I. cardiac output index Table 5 Cardiac catheter oxygen saturation study results SVC superior vena cava, RA right atrium, RV right ventricle, PA pulmonary artery, PCW pulmonary capillary wedge, Ao aorta Table 6 Operative and postoperative results in five cases of coronary sinus atrial septal defect The morphology of URCS was classified as reported by Kirklin and Barratt-Boyes as follows: type I, completely unroofed with PLSVC; type II, completely unroofed without PLSVC; type III, partially unroofed midportion; and type IV, partially unroofed terminal portion.3 URCS unroofed coronary sinus syndrome, PLSVC persistent left superior vena cava, AXC aortic cross-clamp, re-AXC repeat aortic cross-clamp, CPB cardiopulmonary bypass, TAP tricuspid annuloplasty, MVP mitral valve repair (plasty), LAAA left atrial appendage amputation, AVR aortic valve replacement, RA right atrium, MAP mitral annuloplasty, POD postoperative day, ICU intensive care unit, HD hemodialysis, CSM cervical spondylotic myelopathy
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This preprint is under consideration at Journal of Cardiothoracic Surgery. A preprint is a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Case report
Haruhiko Sugimori
New Tokyo Hospital
Corresponding Author
ORCiD: https://orcid.org/0000-0002-9920-119X
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Under Revision
Version 1
Posted 04 Mar, 2021
No community comments so far
Editorial decision: Major revision
On 04 Apr, 2021
Review #2 received
Received 19 Mar, 2021
Review #1 received
Received 12 Mar, 2021
Reviewer #2 agreed
On 01 Mar, 2021
Review #? received
Received 27 Feb, 2021
Reviewer #1 agreed
On 27 Feb, 2021
Reviewers invited
Invitations sent on 26 Feb, 2021
Editor assigned
On 23 Feb, 2021
Submission checks complete
On 23 Feb, 2021
Editor invited
On 23 Feb, 2021
First submitted
On 21 Feb, 2021
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
Subject Areas
Cardiothoracic Surgery
License:
This work is licensed under a CC BY 4.0 License. Read Full License
Background: Isolated coronary sinus atrial septal defect (ASD) is defined as a coronary sinus unroofed in the terminal portion without a persistent left superior vena cava and other anomalies. This defect is rare and part of a wide spectrum of unroofed coronary sinus syndromes. Recently, several reports have described this finding. We searched the hospital’s database to determine the incidence of this defect, and to raise awareness of this condition, we discussed the findings from five patients with coronary sinus ASD who underwent surgical repair.
Case presentation: The patients were three women and two men with an age range of 63–77 years. All patients underwent transthoracic echocardiography and computed tomography, and one underwent magnetic resonance imaging. In two patients, the defect was found unexpectedly intraoperatively; left-to-right shunting was apparent in the other three patients preoperatively. The pulmonary-to-systemic blood flow ratio ranged from 1.42 to 3.1 following cardiac catheterization, and oxygen saturation step-up was seen on the right side of the heart. Valvular regurgitation was seen in 4/5 patients with the mitral, tricuspid, and aortic valves involved in different combinations and to different degrees. Right atrial and ventricular dilation were seen in 4/5 patients; three patients had left atrial dilation. Three patients experienced atrial fibrillation, and one of these also experienced paroxysmal ventricular contractions. All patients underwent surgical repair, and some underwent multiple procedures. One patient who had previously undergone kidney transplantation died approximately 1 year postoperatively; the remaining four patients are currently experiencing good activities of daily living without symptoms.
Conclusions: Coronary sinus ASD (Kirklin and Barratt–Boyes type IV unroofed coronary sinus syndrome) comprised 1.3% of adult congenital heart surgeries and 0.07% of adult open-heart surgeries in our hospital from 1999 to 2019. Our hospital performs cardiac surgery mainly for patients with acquired cardiac disease, and coronary sinus ASD is rare. Early diagnosis and, in symptomatic patients (especially those with blood access shunts, which may overload the heart), early surgical repair are important. The poorly prognostic case in our series is noteworthy, as similar cases have not been reported previously.
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