Evolution of Interventricular Septal Hematoma: Echocardiographic Diagnosis.

•IVSH is a rare complication after cardiac procedures.•It results from myocardial infarction, coronary intervention, and/or direct trauma.•It is associated with morbidity, mortality, and extracorporeal membrane oxygenation support.•Echocardiography aids in diagnosis and follow-up of IVSH.•Spontaneous fistula between the septal hematoma and RV led to decompression and recovery. Interventricular septal hematoma (IVSH) is a rare complication that has been described after several types of surgical procedures including patch closure of a ventricular septal defect (VSD),1Drago M. Butera G. Giamberti A. Lucente M. Frigiola A. Interventricular septal hematoma in ventricular septal defect patch closure.Ann Thorac Surg. 2005; 79: 1764-1765Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar percutaneous coronary artery intervention,2Abdel-Karim A.R. Vo M. Main M.L. Grantham J.A. Interventricular septal hematoma and coronary-ventricular fistula: a complication of retrograde chronic total occlusion intervention.Case Rep Cardiol. 2016; 2016: 8750603PubMed Google Scholar and internal mammary artery grafting to the left anterior descending coronary artery.3Ettles D.F. Firth N. Nair R.U. Williams G.J. Fatal acute left ventricular outflow obstruction due to interventricular septal haematoma—diagnosis by transoesophageal echocardiography.Eur Heart J. 1989; 10: 479-481Crossref PubMed Scopus (13) Google Scholar Often IVSH causes hemodynamic compromise and is associated with significant morbidity and mortality.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Commonly reported complications of IVSH include myocardial rupture, ventricular dysfunction, ventricular outflow tract obstruction, and abscess formation. The hematoma leading to coronary cameral fistula has been previously reported after stent placement in a coronary artery.2Abdel-Karim A.R. Vo M. Main M.L. Grantham J.A. Interventricular septal hematoma and coronary-ventricular fistula: a complication of retrograde chronic total occlusion intervention.Case Rep Cardiol. 2016; 2016: 8750603PubMed Google Scholar We present a unique case of IVSH after closure of a VSD with development of a coronary fistulous communication associated with decompression of the hematoma into the right ventricle (RV) cavity and a favorable clinical outcome. A 5-month-old, 3.8 kg male was born at 34 weeks of gestation with a large perimembranous VSD diagnosed on an echocardiogram performed at 10 days of life for evaluation of heart murmur and tachypnea. The patient had symptoms of heart failure evidenced by failure to thrive requiring hospitalization and nasogastric tube feeding. His weight, height, and body mass index were less than the first percentile for age and gender, and he had only gained 6 g per day despite feeding on 30 Kilocalorie per ounce formula with medium chain triglycerides oil. On physical examination, there was mild tachypnea, a grade 2 holosystolic murmur heard with maximum intensity at the left lower sternal border, wide splitting of the second heart sound, and hepatomegaly. He was medically managed with oral furosemide twice daily. Preoperative echocardiogram (Figure 1) demonstrated a large perimembranous VSD with left to right shunt, dilated left atrium, and normal biventricular systolic function. The patient was referred for surgical closure. Surgery was complicated by a moderate residual VSD after autologous pericardial patch closure; hence, a second bypass run was required. The entire VSD patch was taken down and redone using a Dacron patch through the septal leaflet of the tricuspid valve. The postoperative transesophageal echocardiogram (TEE) showed a hypokinetic interventricular septum, no residual VSD, moderate tricuspid valve regurgitation (TR), and normal biventricular systolic function (Video 1). After chest closure in the operating room, there was systemic hypotension with suspected cardiac tamponade. Transthoracic echocardiogram (TTE) demonstrated an underfilled/compressed RV with severe systolic dysfunction, moderate left ventricular systolic dysfunction, and a hypokinetic, echo-bright ventricular septum with a small hypoechoic space suggestive of IVSH measuring 0.75 × 0.15 cm (Figure 2A, Video 2). Surgical exploration revealed a mild-moderate thrombus in the pericardial space. Hemodynamics improved immediately after reopening the sternum. Despite the lack of significant hematoma in the pericardium, reopening of the sternum led to decompression and better filling of RV resulting in improved cardiac output. Systolic and presumed diastolic dysfunction after two runs of cardiopulmonary bypass and IVSH caused the clinical decompensation. Epicardial echocardiogram revealed thick interventricular septum and small hypoechoic space (Video 3). Four hours after leaving the operating room, the patient decompensated with a hypotensive arrest requiring resuscitation. On postoperative day (POD) 1, the IVSH increased in size (1.1 × 0.35 cm) and developed a fistulous connection into the RV, and biventricular systolic function was moderately depressed (Figure 2B, Videos 4 and 5). On POD 2, the fistulous connection was noted from the left anterior descending artery to the IVSH with diastolic flow, and it ultimately drained into the RV cavity during systole (Figure 2C and 3, Video 6, Video 7, Video 8). There was improvement in TR and biventricular systolic function (mildly decreased; Video 6, Video 7, Video 8). The patient had had 2:1 atrial flutter on POD 0 and accelerated junctional rhythm requiring AAI pacing. By POD 3, arrhythmias had resolved, the patient was weaned off low-dose epinephrine, the patient was hemodynamically stable with normal biventricular systolic function, and delayed sternal closure was performed (Video 9). The IVSH decreased in size (0.6 × 0.2 cm) by POD 18 (Video 10, Video 11, Video 12). The patient was discharged home on POD 20. Three months after the surgery, the echocardiogram demonstrated complete resolution of IVSH with normal biventricular systolic function and trivial TR (Videos 13 and 14).Figure 3Pulse-wave Doppler tracing of fistula from the IVSH demonstrating systolic flow into the RV.View Large Image Figure ViewerDownload Hi-res image Download (PPT) We present the echocardiographic features of a postoperative IVSH related to patch closure of a VSD that developed a fistulous connection to the RV cavity and subsequently resolved. There is currently a lack of consensus on the etiology and management of IVSH. The literature is limited to a few case reports and one case series of 12 patients. In the adult literature, IVSH has been described after myocardial infarction, percutaneous coronary artery intervention, coronary artery bypass graft procedures, or trauma. In the pediatric literature, IVSH has been reported following procedures such as isolated VSD closure, VSD closure along with repair of interrupted aortic arch, coarctation of aorta, transposition of great arteries, and repair of tetralogy of Fallot as well as after relief of pulmonary atresia/critical pulmonary stenosis with intact ventricular septum.5Jensen R. Burg P. Anderson C. Garabedian C. Garabedian H. Siwek L. et al.Postoperative ventricular septal hematoma: natural history of two pediatric cases.J Thorac Cardiovasc Surg. 2007; 133: 1651-1652Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar, 6Mart C.R. Kaza A.K. Postoperative dissecting ventricular septal hematoma: recognition and treatment.ISRN Pediatr. 2011; 2011: 534940Crossref PubMed Google Scholar, 7Jacobs S. Rega F. Vlasselaers D. Gewillig M. Meyns B. Dealing with a septal hematoma after switch operation with ventricular septal defect closure.Heart Surg Forum. 2010; 13: E263-E264Crossref PubMed Scopus (7) Google Scholar, 8Zhu J. Liu H. Zhang J. Feng X. Wu S. Mei J. et al.Interventricular septal hematoma after congenital cardiac surgery.Ann Thorac Surg. 2013; 95: 2171-2173Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar, 9Bailey F.J. Jivanji S.G. Kostolny M. Postoperative interventricular septal haematoma following tetralogy of Fallot repair and perimembranous ventricular septal defect repair.Interact Cardiovasc Thorac Surg. 2017; 24: 296-298PubMed Google Scholar, 10Suteu C.C. Muntean I. Benedek T. Toganel R. Giant dissecting ventricular septal haematoma associated with critical congenital heart disease.Interact Cardiovasc Thorac Surg. 2016; 23: 837-838Crossref PubMed Scopus (2) Google Scholar The etiology is speculated to be due to the insult to the septal perforator branches of the left anterior descending artery during the placement of the VSD patch, specifically the first septal perforator branch, which is in close proximity to the anterior rim of the VSD. This results in subendocardial bleeding, and further expansion of the hematoma occurs due to anticoagulation.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar,11Yamazawa H. Takeda A. Nakajima H. Tachibana T. Aoki M. Interventricular septal hematoma following repair of a ventricular septal defect.J Card Surg. 2017; 32: 390-393Crossref PubMed Scopus (3) Google Scholar However, this theory does not hold true in two reported cases where IVSH developed without VSD closure, one following relief of pulmonary atresia/critical stenosis with intact ventricular septum and the other following repair of total anomalous pulmonary venous return and atrial septal defect closure. The pathogenesis in the former case is thought to be due to hypertensive right ventricular pressures associated with endocardial fibroelastosis and coronary artery ischemia, and in the latter case due to possible high cardioplegia perfusion pressure leading to impaired myocardial perfusion.10Suteu C.C. Muntean I. Benedek T. Toganel R. Giant dissecting ventricular septal haematoma associated with critical congenital heart disease.Interact Cardiovasc Thorac Surg. 2016; 23: 837-838Crossref PubMed Scopus (2) Google Scholar,12Bernasconi A. Cavalle-Garrido T. Redington A. Spontaneous intraoperative ventricular haematoma in a neonate.Heart. 2007; 93: 898Crossref PubMed Scopus (6) Google Scholar Jegatheeswaran et al.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar reported a series of 12 pediatric patients who developed IVSH after VSD closure. They identified that those under 2 months of age and median weight of 3.4 kg may be at higher risk of IVSH likely attributed to the difficulty in visualization of the defect.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Among their 12 cases, 75% developed IVSH after a single cardiopulmonary bypass, while the remainder of the cases occurred following two bypass runs. Complications of IVSH include decreased right and left ventricular systolic and diastolic function, myocardial rupture, thrombus formation, abscess formation, right and left ventricular outflow tract obstruction,2Abdel-Karim A.R. Vo M. Main M.L. Grantham J.A. Interventricular septal hematoma and coronary-ventricular fistula: a complication of retrograde chronic total occlusion intervention.Case Rep Cardiol. 2016; 2016: 8750603PubMed Google Scholar conduction system abnormalities including heart block, junctional tachycardia, cardiac tamponade, requirement of extracorporeal membrane oxygenation support, and risk for multiorgan dysfunction and mortality.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar The mortality reported in the adult literature is higher, ranging up to 78%, especially in those managed convervatively.13Vargas-Barron J. Roldan F.J. Romero-Cardenas A. Molina-Carrion M. Vazquez-Antona C.A. Zabalgoitia M. et al.Dissecting intramyocardial hematoma: clinical presentation, pathophysiology, outcomes and delineation by echocardiography.Echocardiography. 2009; 26: 254-261Crossref PubMed Scopus (39) Google Scholar In comparison, the mortality reported in the pediatric literature is lower at 33% (in 12 pediatric patients after VSD closure); 16% (two cases) needed an intervention to drain the hematoma.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Our patient's initial postoperative TEE did not demonstrate IVSH; however, the TTE in the operating room performed 2 hours later for acute decompensation showed the first sign of IVSH. This is consistent with the reported literature that only 55% (6 of 11 patients) of IVSH were discovered intraoperatively. The average time to diagnosis was 10.1 ± 7.9 hours.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Echocardiography is the key investigation in the diagnosis and follow-up of IVSH. Characteristic features include echo bright, thickened interventricular septum with hypoechoic space. The IVSH can rapidly increase in size postoperatively, hence close follow-up is warranted. Development of fistulous connections to the coronary artery or the ventricular cavity and/or development of right or left ventricular outflow tract obstruction can be identified using two-dimensional (2D), color, and Doppler echocardiography. In addition, the literature has reported on the utility of contrast echocardiography, 2D speckle-tracking echocardiography, three-dimensional echocardiography, cardiac catheterization, and magnetic resonance imaging on diagnosing and evaluating IVSH. Speckle-tracking echocardiography demonstrates decreased myocardial strain in interventricular septal segments with normal strain in the other LV segments.14Grohmann A. Elgeti T. Eddicks S. Knebel F. Rutsch W. Melzer C. et al.Interventricular septum hematoma during cineventriculography.Cardiovasc Ultrasound. 2008; 6: 4Crossref PubMed Scopus (4) Google Scholar Three-dimensional echocardiography demonstrates regional akinesia of the ventricular septum.15Etman I. Abdelgawad H. P178 Dissecting ventricular septal hematoma; a rare complication of acute myocardial infarction.Eur Heart J Cardiovasc Imaging. 2020; 21: jez319.049Crossref Google Scholar In a rare case of spontaneous IVSH with endocardial fibroelastosis, cardiac magnetic resonance imaging in addition to echocardiography was useful in myocardial tissue characterization.16Zhang N. Hu Q. Li Y. Liu J. Xu L. Sun Z. Spontaneous interventricular septum dissecting hematoma with endocardial fibroelastosis: imaging, diagnosis, surgical therapy and 6-year follow-up outcomes.Quant Imaging Med Surg. 2020; 10: 878-882Crossref PubMed Google Scholar Ventricular cine angiography demonstrated accumulation of contrast within the IVSH14Grohmann A. Elgeti T. Eddicks S. Knebel F. Rutsch W. Melzer C. et al.Interventricular septum hematoma during cineventriculography.Cardiovasc Ultrasound. 2008; 6: 4Crossref PubMed Scopus (4) Google Scholar and identified injury to the first septal perforator artery.2Abdel-Karim A.R. Vo M. Main M.L. Grantham J.A. Interventricular septal hematoma and coronary-ventricular fistula: a complication of retrograde chronic total occlusion intervention.Case Rep Cardiol. 2016; 2016: 8750603PubMed Google Scholar The management of the IVSH is often determined by its size and hemodynamic sequalae. Treatment options include conservative management, surgical drainage or needle aspiration of the IVSH. Support with extracorporeal membranous oxygenation has been recently described as a potential successful bridging therapy for hemodynamically unstable patients until the hematoma resolves.4Jegatheeswaran A. Cohen M.S. Gaynor J.W. Mascio C.E. Spray T.L. Fuller S. Extracorporeal membrane oxygenation as a novel management strategy for interventricular septal hematoma following ventricular septal defect repair.J Thorac Cardiovasc Surg. 2020; 159: 1936-1940Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar The mean time for resolution of the IVSH is reported to be variable at 20 ± 185 days. In one case series, 75% of pediatric patients had recovery of ventricular function. In our patient, the unique course of development of fistulous drainage between the IVSH and RV cavity resulted in decompression of the hematoma and likely contributed to spontaneous and early recovery. To our knowledge, this is the first report describing this sequence of echocardiographic changes in the evolution of postoperative IVSH with the development of a fistulous connection between the IVSH and the RV cavity and resolution of the IVSH. We highlight the utility of echocardiography in diagnosis and serial monitoring to evaluate the progress of IVSH. Although rare, IVSH is a serious complication after surgical repair of VSD that is often overlooked in both adult and pediatric cases. Early recognition of this phenomenon is important as it is associated with high morbidity and mortality. Complications such as ventricular dysfunction, myocardial rupture, abscess formation, right or left ventricular outflow tract obstruction, and cardiac tamponade should be ruled out. In our patient, IVSH was associated with severe low cardiac output state, hypotensive arrest, and arrhythmia. During follow-up assessment, the IVSH fistulized to the right ventricular cavity leading to decompression and spontaneous recovery of biventricular systolic function, which led to a good clinical outcome. Recognition of this unusual phenomenon avoided an invasive procedure to drain the IVSH. Serial 2D echocardiography with color Doppler is useful in recognition of this sequence of changes.