Fatal hemorrhage from a periumbilical wound: Stabbing or hemorrhage from a caput medusae?

Varices are the main clinical manifestation of portal hypertension, and their bleeding is the predominant cause of mortality from this condition. Periumbilical varices are known as "caput medusae." Reports of their bleeding are rare, with only three fatal cases described in the literature. The antemortem diagnosis is relatively simple, while the postmortem diagnosis is more complex. This paper is the first report of fatal hemorrhage from a caput medusae for which the diagnosis was made postmortem, thanks to a complete diagnostic process including scene and circumstances, medical history, and autopsy with detailed histology. The circumstantial analysis showed the presence of a large amount of blood at the scene, blood which originated from a small abdominal wound; an analysis of the subject's clinical data reported that he was affected by portal hypertension. The autopsy revealed some dilated and convoluted veins in the subcutaneous tissue of the umbilical region; a fistula between these veins and the abdominal wound was detected. The histological study confirmed the presence of periumbilical varices, one of them ruptured and connected with the overlying skin. The cause of death was attributed to a massive hemorrhage generated by a periumbilical varix in a patient affected by portal hypertension.

Death due to a bronchus-pulmonary artery fistula developed 19 years after radiotherapy: A forensic autopsy case report.

Radiotherapy, one of the standard therapies for lung cancer management, may cause severe late complications. In this case report, we describe the forensic autopsy report of a middle-aged man who died from a massive hemoptysis due to a bronchus-pulmonary artery fistula that developed 19 years after radiotherapy. The man, in his 50 s, suddenly developed hemoptysis at home and collapsed. He was in complete remission with no signs of recurrence. Autopsy revealed massive hemorrhage from the bronchus-pulmonary artery fistula, where radiotherapy had been focused. Histopathological findings showed chondronecrosis of the bronchus, disruption of elastic fibers of the pulmonary artery, and marked thickening of the intima of the small arteries around the fistula, which were compatible with radiation reaction. Neither cancer recurrence nor infection was evident. This case suggests that a late complication of radiotherapy should be considered in the differential diagnosis of a patient who was previously received radiotherapy and presents with massive hemoptysis. In such cases, a detailed history on previous therapies and careful examination of the origin of hemorrhage are necessary to determine the cause of death.

Cardiac remodeling secondary to chronic volume overload is attenuated by a novel MMP9/2 blocking antibody.

OBJECTIVE: Monoclonal antibody derivatives are promising drugs for the treatment of various diseases due to their high matrix metalloproteinases (MMP) active site specificity. We studied the effects of a novel antibody, SDS3, which specifically recognizes the mature active site of MMP9/2 during ventricular remodeling progression in a mouse model of chronic volume overload (VO). METHODS: VO was induced by creating an aortocaval fistula (ACF) in 10- to 12-week-old C57BL male mice. The VO-induced mice were treated with either vehicle control (PBS) or with SDS3 twice weekly by intraperitoneal (ip) injection. The relative changes in cardiac parameters between baseline (day 1) and end-point (day 30), were evaluated by echocardiography. The effects of SDS3 treatment on cardiac fibrosis, cardiomyocyte volume, and cardiac inflammation were tested by cardiac staining with Masson's trichrome, wheat Germ Agglutinin (WGA), and CD45, respectively. Serum levels of TNFα and IL-6 with and without SDS3 treatment were tested by ELISA. RESULTS: SDS3 significantly reduced cardiac dilatation, left ventricular (LV) mass, and cardiomyocyte hypertrophy compared to the vehicle treated animals. The antibody also reduced the heart-to-body weight ratio of the ACF animals to values comparable to those of the controls. Interestingly, the SDS3 group underwent significant reduction of cardiac inflammation and pro-inflammatory cytokine production, indicating a regulatory role for MMP9/2 in tissue remodeling, possibly by tumor necrosis factor alpha (TNFα) activation. In addition, significant changes in the expression of proteins related to mitochondrial function were observed in ACF animals, these changes were reversed following treatment with SDS3. CONCLUSION: The data suggest that MMP9/2 blockage with SDS3 attenuates myocardial remodeling associated with chronic VO by three potential pathways: downregulating the extracellular matrix proteolytic cleavage, reducing the cardiac inflammatory responses, and preserving the cardiac mitochondrial structure and function.

Coronary Arterial Abnormalities in Hypoplastic Left Heart Syndrome: Pathologic Characteristics of Archived Specimens.

Palliation of patients with hypoplastic left heart syndrome remains challenging. Although coronary ischemia can be catastrophic, the prevalence and pathologies of anomalies of the coronary arteries remains unknown. We reviewed 119 specimens with the features of hypoplastic left heart syndrome, focusing our attention on the aortic root and the coronary arteries. We found 36 (30%) specimens with the combination of mitral and aortic atresia, 26 (22%) with mitral and aortic stenosis, and 57 (48%) with mitral stenosis combined with aortic atresia. In 29 specimens (24%), the coronary arteries were not located in the center of any sinuses, while in 24 specimens (21%) at least 1 coronary artery was located very proximal to a raphe or commissure, with potential for obstruction. The specimens with combined stenosis were more likely to have eccentric positions of the coronary arteries (11 specimens, 42%), compared to the 3 specimens with combined atresia (9%, P = 0.009). The specimens with combined stenosis were also more likely to have positioning at risk for obstruction (9 specimens, 35%), compared to those with combined atresia (3 specimens, 9%, P = 0.05). Coronary arterial fistulous communications were found in 11 specimens (9%), significantly more frequently in specimens with mitral stenosis and aortic atresia (9 specimens, 16%, P = 0.041). The origins of the coronary arteries in patients with hypoplastic left heart syndrome place them at potential risk for ischemia, with fistulous communications being a particular risk in those with mitral stenosis combined with aortic atresia.

Aortobronchial fistula.

Aortobronchial fistulas (ABFs) are rare but fatal if left untreated. Hemoptysis is the most common symptom of the patients. ABFs may occur after any thoracic aortic lesions or maneuveurs for these lesions. The treatment of ABF can be surgical or thoracic endovascular aortic repair. Thoracic endovascular aortic repair can be a safe and less invasive procedure for the treatment of ABFs. However, ABFs might occur in much shorter time after thoracic endovascular aortic repair than any other etiologies. The prognoses of patients with ABFs are poor with a high morbidity and mortality. The selection of a suitable endovascular graft and avoidance of postinterventional complications might effectively prevent the occurrence of ABFs.

Sudden death with massive hemoptysis from aortobronchial fistula.

Aortobronchial fistula is an uncommon clinical entity, with unpredictable clinical course and significant mortality. Here, we describe a case of fistulous tract between a descending aortic graft and a branch of the left upper lobar bronchus leading to massive hemoptysis causing death for hemorrhagic shock in a 72-year-old man.

Coronary-cameral fistula with double-chambered right ventricle: appearance on cardiac magnetic resonance imaging and 3D printed anatomic modeling.

The present case illustrates cardiac magnetic resonance imaging (MRI) and three-dimensional (3D) printed anatomic model findings of a coronary-cameral fistula (CCF) and double-chambered right ventricle (DCRV). A pregnant woman presented with palpitations and near syncope. A non-contrast cardiac MRI showed CCF connecting to a DCRV. Post-delivery, the patient had a contrast-enhanced MRI and 3D printed anatomic model to better evaluate her aberrant anatomy.

[Anomalous origin of the right coronary artery from the pulmonary artery or coronary artery fistula: when the diagnosis is uncertain]. / Quando la diagnosi può essere incerta: origine anomala dell'arteria coronaria destra dall'arteria polmonare o fistola coronarica?

The anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital coronary anomaly with a reported incidence of approximately 0.002%. Usually, the diagnosis is made by echocardiography leaving computed tomography or angiography only to diagnostic completion in doubtful cases or for interventional procedures.Herein we report a doubtful case of a patient with a diagnosis of coronary fistula between the right coronary and the pulmonary artery that proved to be an anomalous origin of the coronary artery from the pulmonary artery. The patient underwent corrective surgery with translocation of the coronary artery on the aorta.

Sudden Unexpected Death Due to Left Subclavian Artery-esophageal Fistula Caused by Fish Bone.

A 53-year-old woman was admitted to the hospital due to unexpected dizziness and died the following morning. To investigate the cause of death, a forensic autopsy along with histological examination was performed 3 days after her death. The major findings of the autopsy were that a fish bone had pierced the left subclavian artery after perforating the esophagus with 680 mL of blood in the stomach and bloody and tarry contents were present in the intestines, and the cause of death was confirmed to be subsequent hemorrhagic shock. Unfortunately, none of her family realized that she had eaten a fish 4 days before the tragedy until the fish bone was found. The present case is rare and instructive. The histopathological findings of left subclavian artery-esophageal fistula induced by a fish bone can be used as a reference in forensic practice.

Aortoenteric fistula: a rare but critical cause of small bowel bleeding discovered on capsule endoscopy.

A 78-year-old man attended for outpatient capsule endoscopy, to investigate a recent history of unexplained small bowel bleeding. His previous medical history included an abdominal aortic aneurysm repair 6 years ago. Soon after capsule ingestion, he experienced sudden onset abdominal pain and collapsed on hospital grounds. He was rapidly transferred to the emergency department as he was haemodynamically unstable, and a significant per rectum (PR) bleed was found on examination. The patient was quickly stabilised following fluid resuscitation. CT angiography was performed which did not show active bleeding. However, use of the real-time capsule viewer indicated a profuse active jejunal bleed originating from the aortic graft, suggestive of an aortoenteric fistula. The patient underwent emergency endovascular cuff placement, and subsequent endovascular abdominal aortic stent grafting, to good effect.

Spontaneous closure of an iatrogenic coronary artery fistula during recanalization of a chronic total occlusion lesion: A case report.

RATIONALE: Coronary perforation leading to fistula directed to the right ventricle is a rare complication of percutaneous coronary intervention (PCI). The reported outcomes vary from a stable state to rapid deterioration. PATIENT CONCERNS: An 86-year-old man was diagnosed with non-ST elevation myocardial infarction, and arranged to PCI procedure for the chronic total occluded right coronary artery (RCA) after coronary angiography. The guide wire went through the occluded lesion and got to the distal part of the suspected "post lateral artery", which later proved to be in the right ventricle (RV). After dilating with a 2.0 mm balloon, large amount of contrast medium leaked out from the lesion; therefore, we suspected a perforation into the pericardium. INTERVENTION: Protamine was intravenously injected to convert the effect of heparin and the 2.0 mm balloon in diameter was dilated for about 1 h to obstruct the ejected blood flow shunting into the pericardium, but the leakage persisted. Nevertheless, the patient remained stable, and we were unable to detect an effusion in the pericardium. DIAGNOSIS: By analyzing the angiogram and echocardiogram, we found that the contrast did not leak into the pericardium, but into the right ventricle (RV) chamber. An iatrogenic coronary artery fistula (ICAF) from the RCA to the RV was confirmed. We thus terminated the procedure. OUTCOMES: Coronary computed tomography (CT) angiography was performed 2 days after the PCI and no abnormal shunt was found. There was no abnormal Doppler signal in the RV, either. The patient was soon discharged, and there have been no complaints of discomfort during the 10-month follow-up. LESSONS: ICAFs from coronary to the RV always have favorable outcomes. Even like the one in this case that caused medium leakage could seal spontaneously without any additional management. Echocardiography or coronary CT angiography could be chosen as imaging options to follow-up ICAFs.

Two autopsy cases of rupture of the aorta by fistula formation after thoracic endovascular aortic repair and open stent-grafting on aortic arch aneurysm.

The mortality rate of aortic aneurysm/dissection is low in Japan. Two surgical procedures, the thoracic endovascular aortic repair (TEVAR) and the open stent-grafting have contributed much in survival of such aneurysmal patients. We encountered with two autopsy cases of death by aortic rupture with fistula formation after these procedures. Case 1 is an 85-year-old male who had the history of TEVAR for thoracic aorta aneurysm one and a half year before his death. His endovascular stent-graft was composed of a steel endoskeleton consisting of six Z-shape elements while at autopsy, one of the elements locating at the distal part was found inserted deep into the wall of descending aorta, causing aorto-esophageal fistula. Case 2 is an 88-year-old male who had the history of open stent-grafting for aortic aneurysm eight years ago. At autopsy, the stent-graft was found detached from aorta at its lesser curvature, causing gap formation between the aorta and the stent. Six Z-shaped stent elements, the parts of stent-graft, were found separated from descending aorta and located in the aneurism. Furthermore, three of the separated elements were found inserted deep in the aortic wall, causing aorto-pulmonary fistula. Since aorto-esophageal fistula formation after surgery for aortic aneurysm is very rare in TEVAR and there are no reported cases of death by aorto-pulmonary fistula in the open stent-grafting, these cases are reported here.

A case of Brucella aortitis associated with development of thoracic aortic aneurysm and aortobronchial fistula.

OBJECTIVES: The purpose of this case report is to document the occurrence of granulomatous aortitis complicated by formation of a saccular aneurysm and aortobronchial fistula due to Brucella infection. METHODS: A 65-year-old man with a history of feral swine hunting presented with hemoptysis and was found to have a saccular thoracic aortic aneurysm and associated aortobronchial fistula. The aneurysm underwent operative repair with closure of the aortobronchial fistula. RESULTS: Histopathological examination of the aneurysm wall revealed evidence of granulomatous aortitis. Cultures of the blood and aortic wall tissue were positive for Brucella suis. CONCLUSIONS: Although rare, Brucella infection should be considered in the differential diagnosis of aortic aneurysm with granulomatous aortitis.

Antegrade approach for closure of distal and torturous congenital coronary artery fistula: A case report.

RATIONALE: Coronary artery fistulae (CAF) are uncommon heart lesions, but they are the most frequent significant hemodynamic congenital coronary anomaly. Transcatheter closure is an alternative treatment and a variety of techniques and devices were reported to use for the closure of congenital coronary fistulae. PATIENT CONCERNS: We reported a 5-year-10-month-old boy who was diagnosed as CAF at birth. Follow-up transthoracic echocardiography showed persistent CAF and progressed dilatation of the left coronary artery. DIAGNOSES: Coronary angiography showed torturous coronary vessel and CAF at the distal segment of the left circumflex artery (LCX). INTERVENTION: Antegrade approach with Amplatzer Vascular Plug was used due to the retrograde approach failed and showed a good result. OUTCOMES: During a 3-year follow-up period, the patient showed no symptoms and demonstrated good exercise ability. Follow-up coronary angiography showed the Amplatzer vascular plug was located at an adequate position without a residual shunt from the CAF, and the diameter of the left coronary artery regressed when compared to previous angiography results. LESSONS: The antegrade approach is a feasible method for closing CAF if torturous vessels are noted and fistulae occur at the distal portion of the coronary artery. A large randomized study or case series are needed to investigate the comparison of antegrade and retrograde method for the closure of CAF.

Coronary artery fistula with associated Takotsubo cardiomyopathy: a case report.

BACKGROUND: Coronary artery fistula, first described by Krause in 1865, is an abnormal communication between the coronary artery and one of the four chambers of the heart or one of the great vessels. The communications are often congenital but may also be acquired from trauma or invasive cardiovascular procedures. Half of the cases present with angina pectoris whereas the remaining half are incidentally detected on echocardiogram or angiogram performed for an unrelated reason. Takotsubo cardiomyopathy or stress-induced cardiomyopathy is characterized by transient left ventricular dysfunction with minimal elevation of cardiac biomarkers in the absence of underlying coronary artery disease. Almost 90% of reported patients are postmenopausal women with a history of recent emotional or physical stress. CASE PRESENTATION: We report an unusual case of a 64-year-old Hispanic woman presenting with typical symptoms suggestive of acute coronary syndrome after an extreme familial conflict. There was mild troponin elevation. Cardiac catheterization revealed microfistulas originating from the third portion of the left anterior descending artery draining to the left ventricular cavity. The ventriculogram demonstrated the apical ballooning. We postulate that high local concentration of catecholamine triggered by stress resulted in angina pectoris due to worsening coronary steal from the coronary fistula. Also, the stress-induced adrenergic stimulation unmasked the classical akinetic apex and apical ballooning characteristic of Takotsubo cardiomyopathy. CONCLUSIONS: This case report highlights the rare but important association between two uncommon conditions. To the best of our knowledge, only one similar case has been reported describing a patient with microfistulas to left ventricular cavity and concurrent Takotsubo cardiomyopathy.

Histopathology of Giant Coronary Artery Aneurysm Associated with Coronary Artery Fistula.

Giant coronary artery aneurysms related to coronary fistula are rare, and the precise mechanisms by which they occur are unknown. We present a case of giant coronary artery aneurysm of the left coronary artery to the pulmonary artery fistula with a lack of internal and (or) external elastic lamina and medial degeneration.

Roles of venovenous anastomosis and umbilical cord insertion abnormalities in birthweight discordance in monochorionic-diamniotic twin pregnancies without twin-twin transfusion syndrome.

AIM: We evaluated risk factors for birthweight discordance in monochorionic diamniotic (MCDA) twin pregnancies without twin-twin transfusion syndrome (TTTS). METHODS: We investigated all MCDA twin placentas injected with colored dye at our institution between 2007 and 2015. We excluded pairs of twins with TTTS, fetal demise, or severe fetal malformation. All pairs of twins were assigned to the discordant group (birthweight discordance ≥ 25%) or the concordant group (birthweight discordance < 25%). In each pair of twins, we described vascular anastomoses as either arterioarterial, venovenous (VV), or arterial-venous, and abnormal umbilical cord insertion as either marginal or velamentous. We also recorded placental sharing discordance. RESULTS: A total of 150 placentas were analyzed. The incidence of VV anastomosis in the discordant group (40%) was significantly higher than that in the concordant group (12%, P = 0.005). Unilateral abnormal umbilical cord insertion was significantly more common in the discordant group (85%) than in the concordant group (38%, P < 0.001). Placental sharing discordance was seen more frequently in the discordant group than in the concordant group. Multiple logistic analysis revealed that VV anastomosis (odds ratio: 4.7; 95% confidence interval: 1.2-18.6, P < 0.01) and unilateral abnormal umbilical cord insertion of the smaller twin (odds ratio: 5.7; 95% confidence interval: 1.4-22.9, P < 0.01) were independent risk factors for birthweight discordance. CONCLUSION: VV anastomoses and unilateral abnormal umbilical cord insertion of the smaller twin are independent risk factors for birthweight discordance in MCDA twin pregnancies without TTTS.