Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study.

OBJECTIVE: To investigate pregnancy outcomes, especially the risk of pregnancy-related aortic dissection (AD), in patients with Marfan syndrome (MFS) after prophylactic aortic root replacement (ARR). DESIGN: Retrospective case series study. SETTING: Tertiary perinatal care centre at a university hospital. POPULATION: Pregnant women fulfilling the revised Ghent nosology (2010) criteria for MFS who were managed at our institute. METHODS: The pregnancy outcomes of all patients with MFS managed at our institute between 1982 and September 2016 were reviewed retrospectively based on medical records. MAIN OUTCOME MEASURES: Obstetrical management and complication including the incidence of AD throughout the peripartum period. RESULTS: Among 22 patients (28 pregnancies) who had been managed as potential MFS or related disorders, 14 (17 pregnancies) fulfilled the revised Ghent nosology (2010) criteria for MFS and were enrolled in this study. Five patients (five pregnancies) had received ARR before conception: three (60%) developed type B aortic dissection [AD(B)] during the peripartum period, compared with only one of 10 patients (12 pregnancies) without ARR (P < 0.05, Chi-square test). CONCLUSIONS: Our study results suggest that MFS patients after prophylactic ARR are still at high risk of AD(B) during the peripartum period. Careful pre-pregnancy counselling and multidisciplinary care throughout the peripartum period are essential for the management of MFS, even after surgical repair of an ascending aortic aneurysm. TWEETABLE ABSTRACT: MFS patients after prophylactic ARR are still at high risk of type B aortic dissection during the peripartum period.

Rapidly growing primary cardiac leiomyosarcoma: report of a case.

Primary cardiac leiomyosarcomas are very rare. A 19-year-old man was admitted to a local hospital with dyspnea and hemoptysis. He was later transferred to our hospital because of his worsening dyspnea. An enhanced chest computed tomography scan demonstrated a large mass in the left atrium. A transthoracic echocardiogram showed a large mobile mass in the left atrium. The tumor was totally resected. The pathohistological examination showed leiomyosarcoma. The tumor rapidly recurred. and a second and third operation were performed. After the third operation, the patient was treated with radiotherapy. There was no local recurrence but multiple distant metastases were found 2 months after completion of radiation therapy.

Disruption in the intrathoracic trachea due to blunt trauma.

Intrathoracic tracheal disruption by blunt trauma is rare and potentially life threatening. Here report 3 cases of intrathoracic tracheal disruption due to blunt trauma. Two cases, each 43 year old, involved an unrestrained male driver who suffered a head-on crash, while the other, 63 year old, involved a male who suffered compression. Chest roentgenograms on admission showed remarkable deep cervical and mediastinal emphysema in Cases 1 and 2 and mediastinal emphysema alone in Case 3. Bronchoscopy revealed disruption in the trachea. Primary repair was performed through a right posterolateral thoracotomy in Cases 1 and 3 and through a median sternotomy in Case 2. In all cases the postoperative course was uneventful.

Intrapulmonary sequestration with arterial supply from the left internal thoracic artery: a case report.

Pulmonary sequestration is uncommon in the upper lobe. Its arterial supply from the internal thoracic artery is very rare. Reported here is a case of a 20-year-old male whose presenting symptom was recurrent pneumonia. Helical computed tomography (CT) and three-dimensional reconstruction images showed that aberrant arteries arising from the left internal thoracic artery were supplying the area of sequestration and draining into the pulmonary vein. Selective intra-arterial digital substraction angiogram also showed left internal thoracic artery supplying the area of the sequestration. Helical three-dimensional CT is noninvasive and provides as accurate three-dimensional information of the aberrant vascular supply in intrapulmonary sequestration as the angiography.

Primary cardiac sarcoma: two case reports.

Two case reports of primary cardiac sarcoma, which is uncommon, are presented. The first case, a 38-year-old male, complained of chest tightness. Chest roentgenograms showed enlargement of the cardiac shadow and left pleural effusion. Transthoracic echocardiography and chest magnetic resonance imaging showed a tumor in the right atrium, and pericardial effusion. The tumor involved the right atrial wall and interatrial septum, and was partially resected. Pathohistological examination revealed angiosarcoma. He died 1 month later. The second case, a 19-year-old male complained of dyspnea and orthopnea. Chest roentgenograms showed pulmonary congestion. Transthoracic echocardiography showed a large mobile mass in the left atrium. An emergency operation was performed and the tumor was totally resected. Pathohistological examination demonstrated leiomyosarcoma. The postoperative course was uneventful, but the tumor rapidly recurred. Second and third operations were performed at intervals of 2 months. After the third operation, he was treated with radiotherapy. Local recurrence was not found but multiple distant metastases were found 2 months after completion of radiation therapy.

Terminal warm blood cardioplegia improves the recovery of myocardial electrical activity. A retrospective and comparative study.

OBJECTIVE: The effect of terminal warm blood cardioplegia was analyzed in 191 patients undergoing either coronary artery bypass grafting (CABG) or prosthetic heart valve replacement between Jan. 1990 and Dec. 1995. METHODS: Patients were subdivided into 3 historical cohorts based on the method of myocardial protection: Group A (n = 106), multidose cold crystalloid glucose-potassium cardioplegia, alone; Group B (n = 37), cold crystalloid glucose-potassium cardioplegia plus terminal warm blood cardioplegia, Group C (n = 48), cardioplegia induction with cold crystalloid glucose-potassium cardioplegia, maintenance with multidose cold blood cardioplegia, and terminal warm blood cardioplegia. RESULTS: Of patients undergoing CABG, 5.6% of group A, 70.4% of group B, and 86.7% of group C spontaneously resumed sinus rhythm after aortic declamping, as did 9.1% of group A, 60.0% of group B, and 55.6% of group C of patients undergoing prosthetic heart valve replacement. The incidence of spontaneous recovery was significantly better in groups B and C than in group A (p < 0.05). Over 90% of patients without terminal warm blood cardioplegia developed ventricular fibrillation or tachycardia requiring electrical cardioversion (p < 0.05). Postoperatively, patients without terminal warm blood cardioplegia required temporary epicardial pacing more frequently than those with terminal warm blood cardioplegia (p < 0.05). In patients undergoing prosthetic heart valve replacement, groups B and C, the incidence of postoperative atrial fibrillation was significantly lower than in group A. CONCLUSION: Terminal warm blood cardioplegia thus promoted better postoperative electrophysiological cardiac recovery.

[Blunt rupture of the intrapericardial inferior vena cava: report of two cases].

Blunt rupture of the intrapericardial inferior vena cava is rare. Our experience in recent two cases is presented. Case 1: A 52-year-old male was admitted following a traffic accident. Chest CT demonstrated cardiac tamponade and mediastinal hematoma. Ruptures of the right and left atria across the caudal aspect of the atrial septum, and a separate laceration of intrapericardial IVC were found in the emergency operation. Case 2: A 35-year-old male jumped from the fourth floor of a building. Chest CT revealed descending aortic rupture and the patient was taken to surgery. He died of massive hemorrhage from the aortic rupture. Exploration revealed a rupture of intrapericardial IVC. Recent literatures were reviewed and the mechanism of IVC rupture is discussed.

[Dumbbell type schwannoma of the posterior mediastinum: a report of two cases with different surgical approaches].

Two cases of dumbbell type schwannoma of the posterior mediastinum are reported. Case 1 involved a 16-year-old man and case 2, a 48-year-old woman. They were asymptomatic, and in both cases an abnormal mass shadow of the left mediastinum was incidentally detected on a routine chest roentogenogram. In these cases, CT and MRI revealed that dumbbell type tumors extended to the spinal canal. The operations were performed with thoracic approach cooperated with a neurosurgeon in case 1 and with combined posterior and thoracic approach cooperated with an orthopedic surgeon in case 2. Histopathologic findings indicated all benign schwannomas. The reported 25 cases in Japan sofar are reviewed.

Acute traumatic dissection and blunt rupture of the thoracic descending aorta: A case report.

Rupture of the thoracic aorta following blunt trauma is increasing in incidence and remains a highly lethal injury. Blunt traumatic rupture and acute dissection of the thoracic aorta is very rare. A 50-year-old man involved in a motor vehicle accident on March 3, 1998 was admitted to our hospital one and a half hours following the accident. On admission, he was alert and his hemodynamics were stable. Chest roentgenogram demonstrated a widened mediastinum and multiple left-sided rib fractures. Enhanced chest CT revealed a periaortic hematoma just distal to the isthmus, dissection of the descending thoracic aorta and mediastinal hematoma. With the diagnosis of thoracic aortic rupture and acute DeBakey type IIIB dissection, an emergency operation was performed. Intraoperative transesophageal echocardiogram showed a mobile intimal flap and diminished caliber of the proximal descending aorta. Disruption and dissection of the descending thoracic aorta were found. Prosthetic graft interposition was accomplished with the aid of left atrium-left femoral artery bypass using a centrifugal pump and heparin-coated circuits and a blood collection device for blood conservation. The weak dissected aortic wall was glued and reapproximated with Gelatine-Resorcine-Formol glue. The postoperative course was uneventful.

[Pedicled aortic wall flap for the reconstruction of aortico-ventricular discontinuity--a case report].

A 55-year-old man was operated on urgently for aortic valve endocarditis complicated by an annular abscess at the base of the non-coronary leaflet extending down to the left ventricle. Rapidly progressive heart failure and presence of a friable-appearing vegetation on echocardiography were the indications for urgent operation. Preoperative electrocardiogram showed first degree A-V block. At operation the aortic valve was generally thickened with mild calcification about the commissures. Non-coronary cusp was severely deformed and was nearly detached at its base due to annular abscess formation. Native valve was completely excised and the abscess was debrided. A club or a tongue-shaped pedicled aortic wall flap was prepared to the left of the oblique aortotomy incision with its free end to the distal side of the aorta. The flap was folded inward at its pedicle about 1 cm above the non-coronary annulus and was used to patch the abscess cavity. The aortic valve was replaced with a SJM 23 mm aortic valve prosthesis. The remaining defect of the aortic wall was repaired with a patch of woven-dacron vascular prosthesis. Post-operative hemodynamics of the patient was uneventful and he was given a 6-week course of parenteral antibiotics. He developed complete A-V block during the operation, and a permanent pacemaker was implanted on the 6th postoperative day. One year after operation he has not had recurrent infection and is leading a normal life. When used in the repair of ventriculo-aortic discontinuity created by infective endocarditis, the pedicled aortic wall flap has several advantages. It is easily obtainable and can cover abscesses of almost any shape and size. Unlike aortic root homograft, there is no problem of availability. Technically it is relatively simple to prepare a flap, bring it down through the non-coronary sinus and suture over the abscess. Aortic wall has just appropriate thickness and strength to reinforce the weakened periannular area, and if the debrided cavity is deep, the flap can be folded to obtain double thickness. By using this flap, potentially infected cavity is covered and packed by autologous tissue alone, and the synthetic patch to repair the aortic wall defect is placed well away from the site of possible contamination. In addition to the use in infective endocarditis, the aortic wall flap can predictably be used in the repair of aortic annular defect created by over-zealous removal of calcium in the surgery of calcific aortic stenosis, and in intracardiac patching in aortic annular enlargement operation such as Manouguian operation. To our knowledge, the use of pedicled aortic wall flap for aortic annular reconstruction has not been reported in the literature.

[A case of acute mediastinitis with pyothorax secondary to peritonsillar abscess].

A 41-year-old man was admitted to a hospital elsewhere because of tonsillitis with high grade fever. On the 9th day of hospitalization, the patient complained of dysphagia and dyspnea. A chest X-ray film and a CT scan showed right pleural effusion and pericardial effusion, and he was referred to our hospital. Immediately after admission, he underwent pericardiotomy to relieve cardiac tamponade, and a right thoracic tube was inserted for pyothorax. Next day, mediastinal drainage was accomplished through a cervical incision and a right thoracotomy. Eight drainage tubes were left in place. Cultures revealed alpha-Streptococcus, Neisseria and group F Streptococci. Continuous closed irrigation with diluted Isodine (povidone iodine) solution was performed. The last extubation of the drainage tube was done on the 140th day after operation. He was cured and discharged on the 162nd day after operation. In patients with extensive acute mediastinitis secondary to deep cervical infection, early complete mediastinal drainage via a cervical and a transthoracic incision is essential.

[A case of aortic valve rupture due to blunt chest trauma].

A case of aortic valve rupture due to blunt chest trauma is presented. 19-year-old man was in good health until he sustained traumatic injury in a traffic accident. He was admitted to a hospital elsewhere because of blunt chest trauma and fractures of the bilateral lower extremities. A heart murmur was detected and echocardiogram revealed marked aortic regurgitation. Then he was referred to our hospital. Progressive congestive heart failure developed. He underwent aortic valve replacement with 25 mm SJM prosthesis 9 days after the injury. The noncoronary cusp was found to be ruptured parallel to and 2 mm from its attachment. His postoperative course was uneventful and he is doing well 4 years and 6 months after operation.

[A case of tricuspid regurgitation due to blunt chest trauma].

A case of tricuspid regurgitation due to blunt chest trauma is presented. A 35-year-old man was in good health until he sustained blunt chest trauma in a traffic accident 17 years ago (in 1972). After that easy fatiguability developed. In 1977, slight tricuspid regurgitation was detected, but he was clinically well and no treatment seemed necessary. In October 1989, he was admitted due to right heart failure. The chest X-ray film showed marked cardiomegaly and ECG revealed atrial fibrillation and complete right bundle branch block. Two-dimensional echocardiogram showed a flail anterior leaflet of the tricuspid valve and severe tricuspid regurgitation. In December 1989, he underwent tricuspid valve replacement with Carpentier-Edwards bioprosthesis. The chordae tendineae to the anterior leaflet of the tricuspid valve were ruptured. Furthermore, an artificial cardiac pacemaker was implanted because of slow atrial fibrillation. His postoperative course was uneventful.

[Results of heart valve replacement: especially on the selection criteria for valve prostheses and on a new anticoagulation regimen].

Since March 1981 till the end of 1987, a total of 76 artificial heart valves were implanted in 72 patients. Heart valve prostheses were selected individually according to the patient's needs rather than assigning a single model to every patient. Postoperatively patients were placed on an anticoagulation therapy regimen consisting of reduced-dose warfarin (prothrombin time maintained at 30% of the control) and a small dose (81 mg/day) aspirin. During this period no bioprosthesis was implanted. For aortic valves, Starr-Edwards caged-ball valves were used in 53.1% and St. Jude Medical (SJM) valves in 37.5%. For mitral valves, SJM valves were used in 68%. No mechanical valve failure and no thrombosed valve was encountered. There was one patient-prosthesis missmatch. The incidence of thromboembolism for the entire series was 5/181.2 patient-years. There was 1-2/181.2 patient-years incidence of hemorrhagic complications. These figures were comparable to the ones with conventional warfarin therapy. No intracranial hemorrhage was encountered. Our selection criteria for artificial heart valves and our method of anticoagulation were discussed in detail. We conclude that at present there is no single prosthesis which satisfies all the varying needs of every patient, and the prosthesis which is best suited to that particular patient should be selected. We are generally happy with our current method of postoperative anticoagulation and will continue with this regimen.

[Systemic origin of the sole artery to the basal segments of the left lung without pulmonary sequestration].

A sixty-one year-old man with squamous cell carcinoma of the left upper lobe had an aberrant systemic artery to the left basal segments without pulmonary sequestration. Physical examination revealed neither cardiac murmur nor any sign of heart failure, which was at variance with reported cases in the literature. Chest X-ray film showed no abnormal density suggesting sequestrated lung. Bronchogram disclosed obstruction of the left upper lobar bronchus by the tumor and normal segmental bronchi of the lower lobe. Left pulmonary angiogram showed normal arterial distribution of the left upper lobe and the superior segment of the lower lobe, but the basal segmental arteries were not visualized. The aberrant pulmonary artery arising from the descending aorta was visualized by computed tomography. Following dissection of the abnormal vessel pneumonectomy was performed. Pathological examination of the left basal segments revealed prominent atheromatous changes in the aberrant systemic intrapulmonary artery and irreversible obstructive lesions in its tributaries. These arterial lesions in this patient would have precluded plastic operations such as transfer of the origin of the aberrant vessel to the left pulmonary artery even if other circumstances had been favorable for preservation of the left lower lobe.

[Pure pulmonary atresia complicated by major sinusoidal-coronary artery communication--a case report].

The current treatment of pure pulmonary atresia consists of early initiation of prostaglandins followed by a combination of surgical procedures according to the right ventricular morphology. Major right ventricular sinusoidal-coronary artery communications (SCAC) occasionally present in patients with hypoplastic right ventricle may remain as coronary artery-right ventricular fistulae after right ventricular decompression, which may in turn be the cause for poor surgical results after pulmonary valvotomy in neonates with the SCAC. Herein, a case of this anomaly is reported with a review of literature. A 2-month-old infant underwent an aorto-pulmonary artery shunt operation using Gore-Tex graft for an emergency treatment of pure pulmonary atresia with major SCAC. The catheterization and ventriculography performed 6 months later disclosed suprasystemic right ventricular pressure and more extended SCAC. Then, right ventricular outflow tract was constructed utilizing Gore-Tex patch across the pulmonary valve under cardiopulmonary bypass. The aortico-pulmonary artery shunt was left functioning. After another 6 months, cardiac catheterization and ventriculography were repeated. Right ventricular pressure decreased to the systemic level, right ventricular ejection fraction improved, and the SCAC was not visualized by ventriculography. The decreased inter-ventricular pressure difference and improved right ventricular emptying after the second operation may have played a role in the disappearance of the SCAC. For the neonates with pure pulmonary atresia and sinusoidal-coronary artery communications, we still advocate systemic-pulmonary shunt procedure with concomitant or slightly delayed pulmonary valvotomy as the initial procedure. Unless the tricuspid valve is critically small or stenotic, there may still be a chance for the growth of the right ventricle even in this type of case.