Case Report
An Unusual Case of Severe Stenosis of the Coronary Sinus Ostium in Association With Double Inlet Left Ventricle
World Journal for Pediatric and Congenital Heart Surgery 2014, Vol. 5(3) 473-474 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114525646 pch.sagepub.com
Pip M. Hidestrand, MD1, Edward C. Kirkpatrick, DO1, and Michael E. Mitchell, MD2
Abstract We present a patient with complex single ventricle physiology who was subsequently diagnosed with atresia of the coronary sinus ostium in the setting of myocardial dysfunction following operative palliation. Although a rare cardiac defect, awareness is important as the coronary venous system will often drain to a left superior vena cava (LSVC). If the LSVC is ligated without knowing of this defect, cardiac dysfunction and death can occur. Keywords congenital heart disease, congenital heart surgery, coronary sinus, univentricular heart Submitted October 8, 2013; Accepted January 30, 2014.
Introduction Stenosis/atresia of the coronary sinus ostium is a rare cardiac anomaly estimated to occur in *2% of patients with single ventricle,1 which often goes undetected until surgery. We present a patient with complex single ventricle physiology who was subsequently diagnosed with atresia of the coronary sinus ostium in the setting of myocardial dysfunction following operative palliation.
Case A four-month-old male with a history of double inlet left ventricle, L-transposition of the great vessels, and pulmonary valve stenosis with a single right coronary artery presented for a Damus-Kaye-Stansel (D-K-S) procedure with bidirectional Glenn anastomosis. Prior echocardiogram showed a welldeveloped innominate vein and right superior vena cava without evidence of a distended coronary sinus or left superior vena cava (LSVC). No pre-Glenn cardiac catheterization was performed. Surgical repair was performed at 32 C. Despite routine administration of cold antegrade cardioplegia, there was recurrent early return of cardiac rhythm requiring repeated doses. Additionally, return of blood flow from the coronary artery during D-K-S reconstruction was noted. Postoperative transesophageal echocardiogram showed appropriate coronary flow but markedly decreased left ventricular function. Despite rapid overall clinical improvement, ventricular function continued to decrease on echocardiogram, and the patient had lower than expected oxygen saturations (mid to high 70s) on 1 L of nasal
cannula. Cardiac catheterization was performed and revealed a small persistent LSVC that had not been recognized on previous echocardiograms. The LSVC connected to the coronary sinus with dilation of the coronary venous system and atresia of the ostium (Figure 1). Small communicating channels from the coronary veins were seen draining into the atria. The single coronary artery gave off patent right and left coronary arteries. Consideration was given to return to the operating room for unroofing of the coronary sinus; however, because the patient exhibited improvement and there was adequate decompression through the LSVC, we elected to take a conservative approach. The patient demonstrated continued improvement and ultimately was discharged to home on postoperative day 17 without further sequelae.
Comment Atresia of the coronary sinus is a rare abnormality. Of the 40 cases of atresia of the coronary sinus reported in the literature,1-3 nine 1 Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, WI, USA 2 Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI, USA
Corresponding Author: Pip M. Hidestrand, Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, 9000W Wisconsin Ave, Milwaukee, WI 53226, USA. Email: [email protected]
Downloaded from pch.sagepub.com at GEORGIAN COURT UNIV on May 1, 2015
474
World Journal for Pediatric and Congenital Heart Surgery 5(3)
Figure 1. Superior caval venography in the anterior posterior (A) and lateral (B) projection showing contrast filling into dilated Thebesian veins. Minimal contrast is seen entering the right atrium, indicating a severely stenotic coronary sinus ostium.
had single ventricle physiology, and of these, two had similar anatomy to our case.2 Most cases are associated with persistence of an LSVC, which serves as an egress of the coronary venous blood through the LSVC and into the right atrium.3 In cases of coronary sinus atresia and an LSVC, isolated ligation of the LSVC can cause disruption of coronary venous drainage, resulting in dysfunction and death.4 Failure of antegrade cardioplegia warrants evaluation for coronary sinus atresia/stenosis in all surgical cases. The reason for inadequate cardioplegia in our case was likely due to unrecognized wash out of cardioplegia from the drainage of the LSVC to the coronary sinus. Some have described ligation of the LSVC with concurrent unroofing of the coronary sinus to treat this problem once it is recognized.1,5 We did not recognize this anatomic variant during the operation, and following postoperative catheterization, we chose to leave the LSVC to coronary sinus connection open as it was unobstructed and Glenn pressures were low (7-8 mm Hg). If this had been recognized preoperatively, one approach could have been to snare the LSVC following the antegrade cardioplegia dose to avoid wash out. In addition, consideration would have been given to unroofing the coronary sinus. It could be that early postoperative dysfunction was the direct result of elevated pulmonary artery pressures resulting in elevated Glenn pressures that improved with time thereby decreasing coronary sinus pressures. In our case, this was largely recovered by discharge and almost completely resolved at six-month follow-up.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Salminen JT, Hakala T, Pihkala J, Mattila I, Puntila J, Sairanen H. Coronary sinus orifice atresia with left superior vena cava in patients with univentricular heart. Ann Thorac Surg. 2006;81(5): e16-e17. 2. Filippini LH, Ovaert C, Nykanen DG, Freedom RM. Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections. Heart. 1998;79(5): 509-512. 3. Santoscoy R, Walters HL III, Ross RD, Lyons JM, Hakimi M. Coronary sinus ostial atresia with persistent left superior vena cava. Ann Thorac Surg. 1996;61(3): 879-882. 4. Fulton JO, Mas C, Brizard CP, Karl TR. The surgical importance of coronary sinus orifice atresia. Ann Thorac Surg. 1998;66(6): 2112-2114. 5. Takabayashi S, Shimpo H, Yokoyama K. Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia. Gen Thorac Cardiovasc Surg. 2007;55(5): 197-199.
Downloaded from pch.sagepub.com at GEORGIAN COURT UNIV on May 1, 2015
An Unusual Case of Severe Stenosis of the Coronary Sinus Ostium in Association With Double Inlet Left Ventricle
World Journal for Pediatric and Congenital Heart Surgery 2014, Vol. 5(3) 473-474 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114525646 pch.sagepub.com
Pip M. Hidestrand, MD1, Edward C. Kirkpatrick, DO1, and Michael E. Mitchell, MD2
Abstract We present a patient with complex single ventricle physiology who was subsequently diagnosed with atresia of the coronary sinus ostium in the setting of myocardial dysfunction following operative palliation. Although a rare cardiac defect, awareness is important as the coronary venous system will often drain to a left superior vena cava (LSVC). If the LSVC is ligated without knowing of this defect, cardiac dysfunction and death can occur. Keywords congenital heart disease, congenital heart surgery, coronary sinus, univentricular heart Submitted October 8, 2013; Accepted January 30, 2014.
Introduction Stenosis/atresia of the coronary sinus ostium is a rare cardiac anomaly estimated to occur in *2% of patients with single ventricle,1 which often goes undetected until surgery. We present a patient with complex single ventricle physiology who was subsequently diagnosed with atresia of the coronary sinus ostium in the setting of myocardial dysfunction following operative palliation.
Case A four-month-old male with a history of double inlet left ventricle, L-transposition of the great vessels, and pulmonary valve stenosis with a single right coronary artery presented for a Damus-Kaye-Stansel (D-K-S) procedure with bidirectional Glenn anastomosis. Prior echocardiogram showed a welldeveloped innominate vein and right superior vena cava without evidence of a distended coronary sinus or left superior vena cava (LSVC). No pre-Glenn cardiac catheterization was performed. Surgical repair was performed at 32 C. Despite routine administration of cold antegrade cardioplegia, there was recurrent early return of cardiac rhythm requiring repeated doses. Additionally, return of blood flow from the coronary artery during D-K-S reconstruction was noted. Postoperative transesophageal echocardiogram showed appropriate coronary flow but markedly decreased left ventricular function. Despite rapid overall clinical improvement, ventricular function continued to decrease on echocardiogram, and the patient had lower than expected oxygen saturations (mid to high 70s) on 1 L of nasal
cannula. Cardiac catheterization was performed and revealed a small persistent LSVC that had not been recognized on previous echocardiograms. The LSVC connected to the coronary sinus with dilation of the coronary venous system and atresia of the ostium (Figure 1). Small communicating channels from the coronary veins were seen draining into the atria. The single coronary artery gave off patent right and left coronary arteries. Consideration was given to return to the operating room for unroofing of the coronary sinus; however, because the patient exhibited improvement and there was adequate decompression through the LSVC, we elected to take a conservative approach. The patient demonstrated continued improvement and ultimately was discharged to home on postoperative day 17 without further sequelae.
Comment Atresia of the coronary sinus is a rare abnormality. Of the 40 cases of atresia of the coronary sinus reported in the literature,1-3 nine 1 Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, WI, USA 2 Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI, USA
Corresponding Author: Pip M. Hidestrand, Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, 9000W Wisconsin Ave, Milwaukee, WI 53226, USA. Email: [email protected]
Downloaded from pch.sagepub.com at GEORGIAN COURT UNIV on May 1, 2015
474
World Journal for Pediatric and Congenital Heart Surgery 5(3)
Figure 1. Superior caval venography in the anterior posterior (A) and lateral (B) projection showing contrast filling into dilated Thebesian veins. Minimal contrast is seen entering the right atrium, indicating a severely stenotic coronary sinus ostium.
had single ventricle physiology, and of these, two had similar anatomy to our case.2 Most cases are associated with persistence of an LSVC, which serves as an egress of the coronary venous blood through the LSVC and into the right atrium.3 In cases of coronary sinus atresia and an LSVC, isolated ligation of the LSVC can cause disruption of coronary venous drainage, resulting in dysfunction and death.4 Failure of antegrade cardioplegia warrants evaluation for coronary sinus atresia/stenosis in all surgical cases. The reason for inadequate cardioplegia in our case was likely due to unrecognized wash out of cardioplegia from the drainage of the LSVC to the coronary sinus. Some have described ligation of the LSVC with concurrent unroofing of the coronary sinus to treat this problem once it is recognized.1,5 We did not recognize this anatomic variant during the operation, and following postoperative catheterization, we chose to leave the LSVC to coronary sinus connection open as it was unobstructed and Glenn pressures were low (7-8 mm Hg). If this had been recognized preoperatively, one approach could have been to snare the LSVC following the antegrade cardioplegia dose to avoid wash out. In addition, consideration would have been given to unroofing the coronary sinus. It could be that early postoperative dysfunction was the direct result of elevated pulmonary artery pressures resulting in elevated Glenn pressures that improved with time thereby decreasing coronary sinus pressures. In our case, this was largely recovered by discharge and almost completely resolved at six-month follow-up.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Salminen JT, Hakala T, Pihkala J, Mattila I, Puntila J, Sairanen H. Coronary sinus orifice atresia with left superior vena cava in patients with univentricular heart. Ann Thorac Surg. 2006;81(5): e16-e17. 2. Filippini LH, Ovaert C, Nykanen DG, Freedom RM. Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections. Heart. 1998;79(5): 509-512. 3. Santoscoy R, Walters HL III, Ross RD, Lyons JM, Hakimi M. Coronary sinus ostial atresia with persistent left superior vena cava. Ann Thorac Surg. 1996;61(3): 879-882. 4. Fulton JO, Mas C, Brizard CP, Karl TR. The surgical importance of coronary sinus orifice atresia. Ann Thorac Surg. 1998;66(6): 2112-2114. 5. Takabayashi S, Shimpo H, Yokoyama K. Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia. Gen Thorac Cardiovasc Surg. 2007;55(5): 197-199.
Downloaded from pch.sagepub.com at GEORGIAN COURT UNIV on May 1, 2015
