Surgical management and clinical reflections on acute type A intramural hematoma with a focal intimal tear in an adult with double aortic arch: a case report

BMC Surg. 2026 Jun 30. doi: 10.1186/s12893-026-03680-y. Online ahead of print.

ABSTRACT

BACKGROUND: Double aortic arch (DAA) is a rare congenital aortic arch anomaly that is usually identified in infancy because of symptoms related to a vascular ring. Acute type A intramural hematoma (IMH) with a focal intimal tear in an adult with DAA is extremely rare, and no consensus has been established regarding emergency management.

CASE PRESENTATION: Seventy-three year-old woman presented with burning pain in the throat and suprasternal notch and was initially suspected of having acute coronary syndrome. Computed tomography angiography (CTA) of the whole aorta and supra-aortic vessels demonstrated a double aortic arch, with the right common carotid artery and right subclavian artery originating from the right arch and the left common carotid artery and left subclavian artery originating from the left arch. After preoperative evaluation, emergency surgery was performed through a median sternotomy. Intraoperatively, a focal intimal tear approximately 2 cm in length was identified in the ascending aorta. Given the complex branching anatomy of the double aortic arch, right femoral artery cannulation was used to establish cardiopulmonary bypass in order to minimize invasive manipulation of the arch. Resection of the diseased ascending aorta and graft replacement were performed, and the potential false lumen at the aortic root was obliterated using the adventitial inversion technique. The prosthetic graft was wrapped with bovine pericardium, and the double aortic arch was not addressed during the same operation. The patient experienced recurrent perioperative hypoxemia and was extubated 17 h after surgery following respiratory support, lung-protective management, and anti-inflammatory treatment. She was discharged on postoperative day 12. Approximately 1 month later, she was readmitted with chest pain, and CTA revealed a newly developed dissection in the proximal right aortic arch. The family declined reoperation, and the patient was subsequently lost to follow-up.

CONCLUSIONS: In patients with DAA complicated by acute type A IMH with a focal intimal tear, limited ascending aortic replacement in the emergency setting may reduce surgical trauma; however, it may leave a high-risk residual arch segment and increase the risk of clamp-related injury or insufficient resection margins. Perioperative airway compression caused by the vascular ring should be assessed using imaging, and one-stage or staged reconstruction should be planned according to the patient’s condition. Strict postoperative blood pressure control and close follow-up are essential to reduce the risk of recurrence.

PMID:42380909 | DOI:10.1186/s12893-026-03680-y

Surgical management and clinical reflections on acute type A intramural hematoma with a focal intimal tear in an adult with double aortic arch: a case report

BMC Surg. 2026 Jun 30. doi: 10.1186/s12893-026-03680-y. Online ahead of print.

ABSTRACT

BACKGROUND: Double aortic arch (DAA) is a rare congenital aortic arch anomaly that is usually identified in infancy because of symptoms related to a vascular ring. Acute type A intramural hematoma (IMH) with a focal intimal tear in an adult with DAA is extremely rare, and no consensus has been established regarding emergency management.

CASE PRESENTATION: Seventy-three year-old woman presented with burning pain in the throat and suprasternal notch and was initially suspected of having acute coronary syndrome. Computed tomography angiography (CTA) of the whole aorta and supra-aortic vessels demonstrated a double aortic arch, with the right common carotid artery and right subclavian artery originating from the right arch and the left common carotid artery and left subclavian artery originating from the left arch. After preoperative evaluation, emergency surgery was performed through a median sternotomy. Intraoperatively, a focal intimal tear approximately 2 cm in length was identified in the ascending aorta. Given the complex branching anatomy of the double aortic arch, right femoral artery cannulation was used to establish cardiopulmonary bypass in order to minimize invasive manipulation of the arch. Resection of the diseased ascending aorta and graft replacement were performed, and the potential false lumen at the aortic root was obliterated using the adventitial inversion technique. The prosthetic graft was wrapped with bovine pericardium, and the double aortic arch was not addressed during the same operation. The patient experienced recurrent perioperative hypoxemia and was extubated 17 h after surgery following respiratory support, lung-protective management, and anti-inflammatory treatment. She was discharged on postoperative day 12. Approximately 1 month later, she was readmitted with chest pain, and CTA revealed a newly developed dissection in the proximal right aortic arch. The family declined reoperation, and the patient was subsequently lost to follow-up.

CONCLUSIONS: In patients with DAA complicated by acute type A IMH with a focal intimal tear, limited ascending aortic replacement in the emergency setting may reduce surgical trauma; however, it may leave a high-risk residual arch segment and increase the risk of clamp-related injury or insufficient resection margins. Perioperative airway compression caused by the vascular ring should be assessed using imaging, and one-stage or staged reconstruction should be planned according to the patient’s condition. Strict postoperative blood pressure control and close follow-up are essential to reduce the risk of recurrence.

PMID:42380909 | DOI:10.1186/s12893-026-03680-y

Cervical ectopia cordis in a neonatal camel (Camelus dromedarius): A rare case report from North Kordofan, Sudan

Open Vet J. 2025 Dec;15(12):6805-6808. doi: 10.5455/OVJ.2025.v15.i12.59. Epub 2025 Dec 31.

ABSTRACT

BACKGROUND: Ectopia cordis (EC) is a rare congenital condition in which the heart is partially or completely displaced outside the thoracic cavity. This abnormality is most commonly recognized in humans but has also been observed in dogs, cats, cattle, pigs, and goats. However, it has not been documented in camelids.

CASE DESCRIPTION: This case report describes the case of a neonatal camel calf born in rural Wad Ashana, North Kordofan, Sudan, who presented with EC. The heart was located in its pericardium outside the thoracic cavity, at the most ventral aspect of the cervical region. The heart exhibited a normal morphology and a normal heartbeat. Despite the absence of any additional malformations, the calf died 2 days after delivery.

CONCLUSION: This case is the first documented instance of EC in camels from Sudan and offers important information for veterinary literature on congenital anomalies.

PMID:42376508 | PMC:PMC13313918 | DOI:10.5455/OVJ.2025.v15.i12.59

When an Atrial Septal Defect Is No Longer Silent: A Case Report of a Large Symptomatic Defect Presenting in Adulthood

Cureus. 2026 May 19;18(5):e109183. doi: 10.7759/cureus.109183. eCollection 2026 May.

ABSTRACT

Atrial septal defects (ASDs) are among the most common forms of congenital heart disease diagnosed in adulthood. Ostium secundum defects represent the majority of cases and may remain clinically silent for decades. Delayed diagnosis can lead to progressive right-sided cardiac chamber dilation, pulmonary hypertension, arrhythmias, and heart failure. The choice between percutaneous and surgical closure depends on anatomical characteristics such as defect size and the adequacy of surrounding septal rims. We report the case of a 49-year-old woman with hypertension, type 2 diabetes mellitus, and hyperlipidemia who presented with one week of substernal chest pain, palpitations, and progressive dyspnea on exertion consistent with New York Heart Association (NYHA) functional class III limitation. Physical examination revealed a holosystolic murmur best heard along the tricuspid area with inspiratory accentuation. Transthoracic echocardiography identified a large secundum ASD measuring 35 mm × 22 mm with elevated pulmonary artery systolic pressure and right-sided chamber dilation. Transesophageal echocardiography confirmed a large defect with minimal aortic rim and limited atrioventricular valve rim, precluding percutaneous closure. Cardiac catheterization demonstrated bidirectional shunting with a Qp/Qs ratio of 2.65 and normal pulmonary vascular resistance. The patient subsequently underwent successful surgical ASD closure using a bovine pericardial patch. Her postoperative course was uncomplicated, with complete resolution of symptoms and normalization of pulmonary artery pressures on follow-up echocardiography six months later. This case highlights the potential for large ASDs to remain undiagnosed until adulthood, when progressive right-sided cardiac remodeling leads to symptomatic disease. Early detection through timely echocardiographic evaluation is critical to facilitate timely intervention and may expand eligibility for less invasive percutaneous repair. Prompt recognition and closure of hemodynamically significant ASDs can prevent irreversible cardiopulmonary complications and significantly improve long-term outcomes.

PMID:42326254 | PMC:PMC13277483 | DOI:10.7759/cureus.109183

Cardiac transplantation in situs inversus post-TAPVC repair

Indian J Thorac Cardiovasc Surg. 2026 Jul;42(7):892-896. doi: 10.1007/s12055-026-02183-y. Epub 2026 Feb 19.

ABSTRACT

Cardiac transplantation in situs inversus with dextrocardia is rare and surgically complex, owing to the reconstruction of systemic and pulmonary venous pathways. We present a 21-year-old male diagnosed with complex cyanotic congenital heart disease with situs inversus, dextrocardia, mixed total anomalous pulmonary venous connection (TAPVC), and good ventricular function with single ventricle physiology. He underwent left superior vena cava (LSVC) to left pulmonary artery (LPA) bidirectional (BD) Glenn shunt, with mixed TAPVC repair at 3 years of age. At the age of 21 years, he developed severe restrictive myocardial dysfunction and was not suitable for Fontan completion surgery. Hence, he was considered for orthotopic cardiac transplantation. The donor pericardium and donor heart were harvested with the full length of the superior vena cava (SVC) and the innominate vein. The ascending aorta and the aortic arch were harvested to the maximum possible length. After recipient cardiectomy and taking down the Glenn shunt, the recipient’s right atrium (RA) was constructed into a composite tunnel by suturing the retained right atrial cuff posteriorly with bovine pericardium anteriorly. The donor inferior vena cava (IVC) was anastomosed to this tunnel. The donor right SVC was anastomosed to the recipient LSVC using the donor aortic homograft as an interposition graft. The native pulmonary artery (PA) confluence was anastomosed to the donor PA using donor pericardium to augment the anterior and lateral parts of the conduit, followed by aortic anastomosis. Post-procedure, the patient performed well with acceptable hemodynamics, and the heart occupied a mesocardial position.

PMID:42367276 | PMC:PMC13304018 | DOI:10.1007/s12055-026-02183-y

Cardiac transplantation in situs inversus post-TAPVC repair

Indian J Thorac Cardiovasc Surg. 2026 Jul;42(7):892-896. doi: 10.1007/s12055-026-02183-y. Epub 2026 Feb 19.

ABSTRACT

Cardiac transplantation in situs inversus with dextrocardia is rare and surgically complex, owing to the reconstruction of systemic and pulmonary venous pathways. We present a 21-year-old male diagnosed with complex cyanotic congenital heart disease with situs inversus, dextrocardia, mixed total anomalous pulmonary venous connection (TAPVC), and good ventricular function with single ventricle physiology. He underwent left superior vena cava (LSVC) to left pulmonary artery (LPA) bidirectional (BD) Glenn shunt, with mixed TAPVC repair at 3 years of age. At the age of 21 years, he developed severe restrictive myocardial dysfunction and was not suitable for Fontan completion surgery. Hence, he was considered for orthotopic cardiac transplantation. The donor pericardium and donor heart were harvested with the full length of the superior vena cava (SVC) and the innominate vein. The ascending aorta and the aortic arch were harvested to the maximum possible length. After recipient cardiectomy and taking down the Glenn shunt, the recipient’s right atrium (RA) was constructed into a composite tunnel by suturing the retained right atrial cuff posteriorly with bovine pericardium anteriorly. The donor inferior vena cava (IVC) was anastomosed to this tunnel. The donor right SVC was anastomosed to the recipient LSVC using the donor aortic homograft as an interposition graft. The native pulmonary artery (PA) confluence was anastomosed to the donor PA using donor pericardium to augment the anterior and lateral parts of the conduit, followed by aortic anastomosis. Post-procedure, the patient performed well with acceptable hemodynamics, and the heart occupied a mesocardial position.

PMID:42367276 | PMC:PMC13304018 | DOI:10.1007/s12055-026-02183-y

Cardiac transplantation in situs inversus post-TAPVC repair

Indian J Thorac Cardiovasc Surg. 2026 Jul;42(7):892-896. doi: 10.1007/s12055-026-02183-y. Epub 2026 Feb 19.

ABSTRACT

Cardiac transplantation in situs inversus with dextrocardia is rare and surgically complex, owing to the reconstruction of systemic and pulmonary venous pathways. We present a 21-year-old male diagnosed with complex cyanotic congenital heart disease with situs inversus, dextrocardia, mixed total anomalous pulmonary venous connection (TAPVC), and good ventricular function with single ventricle physiology. He underwent left superior vena cava (LSVC) to left pulmonary artery (LPA) bidirectional (BD) Glenn shunt, with mixed TAPVC repair at 3 years of age. At the age of 21 years, he developed severe restrictive myocardial dysfunction and was not suitable for Fontan completion surgery. Hence, he was considered for orthotopic cardiac transplantation. The donor pericardium and donor heart were harvested with the full length of the superior vena cava (SVC) and the innominate vein. The ascending aorta and the aortic arch were harvested to the maximum possible length. After recipient cardiectomy and taking down the Glenn shunt, the recipient’s right atrium (RA) was constructed into a composite tunnel by suturing the retained right atrial cuff posteriorly with bovine pericardium anteriorly. The donor inferior vena cava (IVC) was anastomosed to this tunnel. The donor right SVC was anastomosed to the recipient LSVC using the donor aortic homograft as an interposition graft. The native pulmonary artery (PA) confluence was anastomosed to the donor PA using donor pericardium to augment the anterior and lateral parts of the conduit, followed by aortic anastomosis. Post-procedure, the patient performed well with acceptable hemodynamics, and the heart occupied a mesocardial position.

PMID:42367276 | PMC:PMC13304018 | DOI:10.1007/s12055-026-02183-y

Pyriphenone modification of glutaraldehyde pretreated bovine pericardium mitigates advanced glycation end products, calcification, and platelet adhesion

Acta Biomater. 2026 Aug;219:224-235. doi: 10.1016/j.actbio.2026.06.040. Epub 2026 Jun 19.

ABSTRACT

Heterograft biomaterials, such as glutaraldehyde-pretreated bovine pericardium (BP), are used to fabricate bioprosthetic heart valves (BHV). BHV durability is limited by structural valve degeneration (SVD), which is caused by either advanced glycation end products (AGE) with associated serum protein deposition, calcification, or both. Vitamin B6 vitamers have been investigated experimentally and clinically for mitigating AGE formation that complicates diabetes. In the present study, we investigated a vitamin B6-based photo-responsive molecule, Pyriphenone (PPh), hypothesizing that PPh could mitigate both BP AGE-serum protein uptake and BP calcification in vivo. PPh was synthesized by reacting pyridoxamine with benzophenone. PPh was optimally soluble in ethanol, and PPh-ethanol solutions were used for all PPh studies. PPh was demonstrated to become covalently and stably attached to BP with exposure to ultraviolet light. PPh-BP demonstrated significantly reduced in vitro AGE deposition and associated serum albumin uptake, versus unmodified BP. PPh-BP showed significant resistance to oxidation in vitro and demonstrated comparable biaxial mechanical properties to unmodified BP. In vitro hydrodynamic testing of trileaflet BHV fabricated from BP-PPh demonstrated no alterations of functionality, compared to unmodified BHV. In vivo 28-day subdermal implants in juvenile rats with either PPh-BP or unmodified BP demonstrated that PPh significantly mitigated AGE and serum albumin uptake, and calcification versus unmodified BP. Ex vivo studies of BP-PPh exposed to human whole blood demonstrated significantly reduced adhesion of platelets and white blood cells versus unmodified BP. In conclusion, PPh-BP mitigates calcification, AGE, and serum albumin uptake and reduces platelet and white blood cell adhesion. STATEMENT OF SIGNIFICANCE: Heart valve disease is highly prevalent, affecting millions. At this time, it can only be treated by either surgical valve repair or replacement of the diseased valve with a prosthesis. Bioprosthetic heart valves, fabricated from heterograft materials, are the most widely used heart valve replacements. However, these devices have poor durability due to calcification and advanced glycation end-product (AGE) deposition, which cause structural valve degeneration. Pyriphenone, the subject of this paper, is a compound synthesized by the authors that is shown in this paper to confer resistance to bioprosthetic valve calcification and AGE deposition.

PMID:42315002 | PMC:PMC13352493 | DOI:10.1016/j.actbio.2026.06.040

Pyriphenone modification of glutaraldehyde pretreated bovine pericardium mitigates advanced glycation end products, calcification, and platelet adhesion

Acta Biomater. 2026 Aug;219:224-235. doi: 10.1016/j.actbio.2026.06.040. Epub 2026 Jun 19.

ABSTRACT

Heterograft biomaterials, such as glutaraldehyde-pretreated bovine pericardium (BP), are used to fabricate bioprosthetic heart valves (BHV). BHV durability is limited by structural valve degeneration (SVD), which is caused by either advanced glycation end products (AGE) with associated serum protein deposition, calcification, or both. Vitamin B6 vitamers have been investigated experimentally and clinically for mitigating AGE formation that complicates diabetes. In the present study, we investigated a vitamin B6-based photo-responsive molecule, Pyriphenone (PPh), hypothesizing that PPh could mitigate both BP AGE-serum protein uptake and BP calcification in vivo. PPh was synthesized by reacting pyridoxamine with benzophenone. PPh was optimally soluble in ethanol, and PPh-ethanol solutions were used for all PPh studies. PPh was demonstrated to become covalently and stably attached to BP with exposure to ultraviolet light. PPh-BP demonstrated significantly reduced in vitro AGE deposition and associated serum albumin uptake, versus unmodified BP. PPh-BP showed significant resistance to oxidation in vitro and demonstrated comparable biaxial mechanical properties to unmodified BP. In vitro hydrodynamic testing of trileaflet BHV fabricated from BP-PPh demonstrated no alterations of functionality, compared to unmodified BHV. In vivo 28-day subdermal implants in juvenile rats with either PPh-BP or unmodified BP demonstrated that PPh significantly mitigated AGE and serum albumin uptake, and calcification versus unmodified BP. Ex vivo studies of BP-PPh exposed to human whole blood demonstrated significantly reduced adhesion of platelets and white blood cells versus unmodified BP. In conclusion, PPh-BP mitigates calcification, AGE, and serum albumin uptake and reduces platelet and white blood cell adhesion. STATEMENT OF SIGNIFICANCE: Heart valve disease is highly prevalent, affecting millions. At this time, it can only be treated by either surgical valve repair or replacement of the diseased valve with a prosthesis. Bioprosthetic heart valves, fabricated from heterograft materials, are the most widely used heart valve replacements. However, these devices have poor durability due to calcification and advanced glycation end-product (AGE) deposition, which cause structural valve degeneration. Pyriphenone, the subject of this paper, is a compound synthesized by the authors that is shown in this paper to confer resistance to bioprosthetic valve calcification and AGE deposition.

PMID:42315002 | PMC:PMC13352493 | DOI:10.1016/j.actbio.2026.06.040

Double-layer repair of a tuberculous ascending aortic aneurysm: a case report and literature review

Int J Surg Case Rep. 2026 Mar 26;138(6):2164-2167. doi: 10.1097/RC9.0000000000000366. eCollection 2026 Jun.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Tuberculous aneurysm of the ascending aorta is extremely rare and life-threatening, particularly in elderly patients. Early recognition and appropriate surgical management are essential to prevent fatal rupture.

CASE PRESENTATION: An 84-year-old man presented with hemoptysis and chest tightness. Computed tomography revealed a saccular aneurysm of the ascending aorta with sternal erosion. During surgery, the aneurysm showed a caseous appearance with dense adhesions and infiltration into adjacent tissue. Frozen section demonstrated granulomatous inflammation suggesting tuberculous aortitis, which was later confirmed by postoperative Xpert MTB/RIF assay. A double-layer aortic repair was performed using an inner bovine pericardial patch and an outer Gelweave graft. The patient recovered well under antituberculous therapy and remained stable at 7-month follow-up.

CLINICAL DISCUSSION: Tuberculous aortitis in elderly patients is challenging due to degenerative aortic wall changes and hypertension. Intraoperative frozen section combined with gross findings played a key role in guiding the surgical strategy. The double-layer repair provided infection control and mechanical durability, consistent with current literature supporting biologic-synthetic hybrid reconstruction in infected aneurysms.

CONCLUSION: A tuberculous aneurysm of the ascending aorta should be considered in atypical cases presenting with hemoptysis. In elderly patients with degenerative aortic walls and hypertension, double-layer repair offers effective infection control and durability when combined with appropriate antituberculous therapy. Early diagnosis and tailored surgical planning are crucial for favorable outcomes.

PMID:42253673 | PMC:PMC13236237 | DOI:10.1097/RC9.0000000000000366