Pulmonary hypertension five years after left pneumonectomy for adenoid cystic carcinoma.

We report the findings in a patient with shortness of breath due to pulmonary hypertension five years after left pneumonectomy. Mediastinal recurrence of an incompletely resected and slowly growing adenoid cystic carcinoma of the left main bronchus had encased the right main pulmonary artery.     

High-pressure pulmonary artery aneurysm and unilateral pulmonary artery agenesis in an adult

The presence of a pulmonary artery aneurysm, major aortopulmonary and coronary-pulmonary collateral vessels, and severe pulmonary hypertension in an adult with unilateral pulmonary artery agenesis and previous patent ductus arteriosus ligation is very rare. A 34-year-old man experienced these conditions. When he was 10 years old, catheterization and angiography revealed right pulmonary artery agenesis, dilation of the main pulmonary artery, multiple collateral vessels extending from the aorta to the right pulmonary system, and a patent ductus arteriosus (shunt ratio, 3.57) that was then ligated; the other conditions were not corrected. This adult patient was in New York Heart Association functional class II; mild central cyanosis was detected only during exercise. The right pulmonary arterial system was seen only at the right hilar area via collateral vessels from the subclavian, bronchial, internal mammary, and intercostal arteries. Angiography revealed collateral vessels from the right and circumflex coronary arteries to the right pulmonary system. The right intraparenchymal pulmonary arterial systems were patent but of small diameter (pulmonary artery pressure, 85 mmHg; ratio of peak right-to-left ventricular pressure, 0.94; peak pulmonary pressure unresponsive to 100% oxygen). Pulmonary vascular resistance was not estimated because of the risk of aneurysmal rupture. We concluded that irreversible pulmonary hypertension had developed (delayed by the patent ductus arteriosus ligation in childhood) and that the patient's only chance for survival was heart-lung transplantation. To sustain the patient until surgery, we administered sildenafil. Herein, we describe the vascular conditions that accompany unilateral absence of the pulmonary artery, and therapeutic methods.     

Iatrogenic esophagobronchial fistula arising in irradiated Barrett's esophagus

A 47-yr-old male underwent a right upper lobectomy for stage IIB bronchoalveolar carcinoma followed by 4600 Gy of irradiation. One year later a fistula formed from an ulcerated region of Barrett's esophagus into the left main bronchus. Bronchotomy repair with onlay patch intercostal muscle flap and esophageal repair with serratus anterior muscle flap plus postoperative esophageal stent placement for stricture resulted in good functional results.     

A case report of multiple pulmonary tumors as a sole manifestation of synovial sarcoma]

A 47-year-old woman was admitted to our hospital for cough and dyspnea. Roentgenologic studies and bronchoscopy revealed multiple lung tumors one of which obstructed the right main bronchus. Right pneumonectomy was performed for the pending obstruction of the trachea. The tumor in the right S1 was found to be protruding into the trachea through the right B1 and the main bronchus in a polypoid fashion. The pathological diagnosis of synovial sarcoma was made on the basis of the characteristic biphasic structure composed of spindle cells and epithelioid cells forming gland-like spaces. Three years and eight months after the pneumonectomy, a nodule in the tendon of the extensor hallucis longus muscle became palpable. It was also a synovial sarcoma pathologically. Synovial sarcoma is a soft tissue sarcoma which usually arises in the extremities. It is very rare for pulmonary metastasis of this tumor to be found while the primary tumor is undetectable.     

Severe stenosis of a long tracheal segment, with agenesis of the right lung and left pulmonary arterial sling

A baby presented at term with respiratory distress was managed with extracorporeal membrane oxygenation. Bronchoscopy revealed tracheal hypoplasia, complete tracheal rings, and agenesis of the right main bronchus. Echocardiography showed a left pulmonary arterial sling arising from the proximal part of the right pulmonary artery. Cardiac catheterization demonstrated abnormal pulmonary vasculature in the left lung which would have prevented survival, even after surgical repair. Diagnostic catheterization was important in delineating the anatomy, and aided in the decision not to proceed with surgical repair.     

Bronchial repair with pulmonary preservation for severe blunt trauma

After a motorcycle accident, a 22-year-old male was diagnosed with multiple rib fractures, left-sided tension pneumothorax, hemothorax and left upper lobe bronchus rupture at its origin. An emergency left thoracotomy revealed an almost complete avulsion of the upper lobe bronchus from the main stem bronchus, a severely damaged pulmonary artery, and a concurrent deep intraparenchymal lower lobe laceration. Since the patient was hemodynamically stable, a decision was made to preserve as much lung as possible. In the event of complex pulmonary trauma, pulmonary preservation is desirable since emergency pneumonectomy is usually associated with high mortality. Early bronchoscopic assessment, careful anesthetic management, and meticulous surgical technique with liberal use of fibrin glue were crucial to successful outcome.     

Severe pulmonary artery involvement of Takayasu arteritis. 3 cases and review of the literature]

Three cases of pulmonary arterial disease were identified out of a cohort of 75 cases of Takayasu's disease. In the first case, pulmonary hypertension considered to be idiopathic caused massive haemoptysis and the death of a 48 year old Spanish woman; autopsy revealed characteristic stenoses of the common carotid arteries. In the second case, haemoptysis led to pneumonectomy in a 23 year old West Indian woman with a diagnosis of agenesis of the right pulmonary artery. Disease of the supraaortic vessels occurred several months later and a retrospective diagnosis of Takayasu's disease of the pulmonary artery was made. In the third case, systematic pulmonary angiography in a 41 year old French woman referred for Takayasu's disease of the supraaortic arteries showed severe stenosis of the right pulmonary artery. She developed severe haemoptysis four years later which led to an attempted balloon angioplasty of the right pulmonary artery and embolisation of a branch of the right coronary artery thought to be the cause of the haemoptysis. This was complicated by posterior wall myocardial infarction but the haemoptysis did not recur thereafter. These three cases and a review of the literature show that the pulmonary lesions of Takayasu's disease occur mostly on the main right pulmonary artery but they may be more distal and involve the pulmonary arterioles. A coronaro-broncho-pulmonary collateral circulation may develop distal to the stenosed segments. The main complications are pulmonary hypertension and massive haemoptysis. Surgery is possible in cases of stenosis of the right main pulmonary artery. The potential role of endoluminal angioplasty is discussed.     

A novel technique of tracheal reconstruction with autologous bronchial pedicle flap

We describe a technique used in 2 patients for resection of a bronchogenic carcinoma arising from the right main bronchus and extending along the lateral wall of the lower trachea without carinal invasion. A right pneumonectomy was performed in the first case, and lower and middle lobes were preserved in the second case after extended upper lobe resection. A flap was mobilized from the non-involved left lateral wall of the right main bronchus, which was left attached to the carina. This was used to close the defect in the lower trachea. An autologous bronchial pedicle flap provides reliable material to repair and reconstruct a massive central airway defect.     

Surgical treatment for tuberculous tracheobronchial stenosis

Thirty-nine patients with bronchial tuberculosis underwent bronchoplastic surgery. The modes of procedures were left upper sleeve lobectomy in 13 patients, sleeve resection of the left main bronchus in 11 patients, sleeve resection of the left main bronchus with concomitant left upper lobectomy in 2 patients, right upper sleeve lobectomy in 6 patients, sleeve resection of the right intermediate bronchus in 2 patients, right sleeve superior segmentectomy of the lower lobe in one patient, sleeve resection of the trachea with concomitant left pneumonectomy in one patient, left lower sleeve lobectomy in one patient, carinal resection with right upper sleeve lobectomy and middle lobectomy in one patient, and dilatation of the left main bronchus with a free skin graft reinforced with a steel wire in one patient. There were one operation death and one operation related death in 1950's. FEV 1.0% of 12 patients whose records of pulmonary function tests performed before and after surgery were available, were increased significantly from 67 +/- 10% to 82 +/- 8% in average. Three patients of laryngotracheal stenosis due to tuberculosis were treated with silicon T-tube. In 2 patients their stenotic lesions were repaired by stenting only, for 36 and 56 months. In one patient, T-tube could not be removed due to laryngeal malacia for more than 12 years.     

Unilateral lung agenesis--detrimental roles of surrounding vessels

Unilateral lung agenesis is a rare congenital defect and could be associated with multiple abnormalities. The patients usually have poor long-term outcomes especially in those with right lung agenesis. We reviewed the 10-year experience in our hospital to describe special clinical features and try to delineate the causes of poor outcomes. From 1995 to 2005, 14 patients less than 18 years of age with unilateral lung agenesis (4 with left agenesis, 10 with right agenesis) were enrolled. Medical records reviewed included diagnosis, presentation, chromosome anomalies, cardiovascular anomalies and interventions, outcomes. We found that the mechanisms of severe airway disease in right lung agenesis included (1) trachea compression by the aortic arch, (2) the presence of "pseudo-ring-sling complex," (3) distended pulmonary artery due to left to right shunt which impinged the only bronchus, and finally (4) the persistent LSVC that restricts the growth of trachea. The etiologies of airway complication in left lung agenesis included anomalous aortic arch compression on trachea and the coexisting heart disease with significant left to right shunt, which impinged on the bronchus. In conclusion, unilateral lung agenesis has frequently associated airway problems due to its surrounding vessels. Satisfactory airway intervention remains challenging. This disease still requires great effort to improve patient outcomes.     

Right lung agenesis with left pulmonary artery sling

We report on a 2-month-old infant girl who had right pulmonary agenesis and an unusual course of the left pulmonary artery. Computed tomography and cardiac catheterization showed that the left pulmonary artery arose from the main pulmonary artery, crossing the midline, and reaching the left lung via an aberrant course between the esophagus and trachea. The coexistence of right pulmonary agenesis and left pulmonary sling is extremely rare. Unlike in other reports, our patient remained symptom-free and in good health, with normal growth and development until age 2 years, when she died from complications during an attack of bronchiolitis caused by respiratory syncytial virus.     

Palliation of left main bronchus compression due to malignant tumor by intubation via a tracheostomy tube.

Intubation of the left main bronchus via a tracheostomy tube was performed in a patient with local recurrence of lung cancer associated with invasion and obstruction of the left main bronchus after right sleeve pneumonectomy. The result was satisfactory not only for preventing asphyxia, but also for maintaining the patency of the airway after extubation of the endotracheal tube.     

The rare association of truncus arteriosus with a cervical double aortic arch presenting with left main bronchial compression

Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4th embryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.     

Autotransplantation for central non-small-cell lung cancer in a patient with poor pulmonary function

Lung resection is the standard therapy for non-small-cell lung cancer confined to the lung. The extent of pulmonary resection is dictated by the location and extent of the tumor and the patient's physiologic ability to tolerate resection. We present the case of a patient who had a large non-small-cell tumor of the lung that involved the right main stem bronchus and the adjacent pulmonary artery; poor pulmonary function precluded a pneumonectomy. Right upper and middle sleeve bilobectomy resection was performed, but reconstruction was not possible due to tethering by the intact lower lobe vein. Therefore, the remaining lower lobe was removed, and the lower lobe vein was divided and reimplanted into the upper lobe pulmonary venous stump. Cephalad advancement and autotransplantation of the lower lobe were then accomplished. These procedures allowed enough mobilization for direct bronchial and arterial reconstruction.     

Management of an intrabronchial rupture of right main pulmonary artery: a case report

We report a case of massive endobronchial hemorrhage following a fistula formation of the right pulmonary artery to the right mainstem bronchus in a 15-year-old girl. The fistula had occurred 39 days after the patient had undergone bilateral lung transplantation for cystic fibrosis. The post-transplantation course was remarkable for bronchial colonization by Aspergillus at the site of right bronchial anastomosis and an episode of spontaneous, self-limited hemoptysis on postoperative day 17. A massive endobronchial hemorrhage during surveillance bronchoscopy occurred 39 days after transplantation. Immediate intervention, including rigid bronchoscopy followed by surgery, was effective in saving the patient. The pathophysiologic hypothesis to explain the fistula of the right pulmonary artery to the right mainstem bronchus probably involves ischemia of the anastomosis with necrosis of the suture zone complicated by endobronchial infection with Aspergillus. Rigid bronchoscopic intervention associated with an excellent medical surgical collaboration was pivotal in successfully rescuing the patient.     

Tracheal agenesis: an unresolved problem

A case of tracheal agenesis diagnosed at birth is discussed in which surgical correction was attempted. Both main bronchi were divided and anastomosis of the main right bronchus to the larynx was attempted, after intrapericardial release of the right lung and severing of the lower pulmonary ligament: bronchoplastic reconstruction of the trachea was impossible. We think that tracheal agenesis, a rare cause of respiratory distress in the newborn, has no suitable therapy today. It is justified to attempt surgical correction if any promising device is at hand or if any doubt exists that an uncorrectable situation is present.     

A case of a gastrobronchial fistula after esophageal reconstruction successfully closed with an intercostal muscle flap

A 63-year-old man with esophageal cancer underwent a subtotal esophagectomy via the thoracolaparotomy approach. Two years after the operation, a gastrobronchial fistula unexpectedly occurred in the right bronchus. After admission, medication including omeprazole and nutritional support administered through an enteral tube improved his general condition, and the gastrobronchial fistula was successfully closed with the seventh intercostal muscle flap. After the operation, sputa were aspirated with a bronchofiberscope through a tracheal incision rather than blindly with a catheter. He was in good condition 10 months after the operation.     

Three-year survival after treatment of a primary pulmonary angiosarcoma

We report an observation of angiosarcoma of the pulmonary artery in a 65-year-old man hospitalized for acute dyspnea revealing a left mass hilaire which arose from the pulmonary artery before the development of obstructive endobronchial extension. The therapeutic sequence associated removal of the lobar bronchus obstruction by interventional endoscopy, chemotherapy using ifosfamide-doxorubicin, complete left pneumonectomy and auxiliary chemotherapy with 2 additional cycles. The patient was free of tumor relapse at nearly 3 years follow-up.     

Congenital absence of the right pulmonary artery with coronary collaterals supplying the affected lung: effect on coronary perfusion

We describe a unique case of congenital absence of a right pulmonary artery presenting in a patient of advanced age and initially misdiagnosed as coronary artery disease. Perfusion of the affected lung was accomplished via anomalous collaterals from right and left circumflex coronary arteries which induced myocardial ischemia, as demonstrated by myocardial perfusion scan. To our knowledge there are only three reports in the international literature, describing unilateral pulmonary artery agenesis with the coronary artery supplying the abnormal lung. All these reports described that the existence of such vessels does not affect the myocardial perfusion. However, here we describe, to our knowledge, for the first time that in a patient with unilateral pulmonary artery agenesis, the existence of collaterals from the coronary arteries to the affected lung can actually have a negative effect in myocardial perfusion and can induce myocardial ischemia. In conclusion, clinicians should be aware of the possibility of undiagnosed cases of unilateral pulmonary artery agenesis presenting with chest pain in advanced age.     

Intercostal-to-pulmonary arterial anastomosis, complicated by high-output heart failure: case report

A patient who had high-output heart failure resulting from anastomosis of the intercostal and bronchial arteries to the right pulmonary artery is reported. The patient was a fifty-one year-old man who was admitted to our hospital with left heart failure. A radionuclide angiocardiogram revealed blood flow from the aorta to the right pulmonary artery. A descending aortogram revealed abnormally dilated right intercostal and bronchial arteries, which formed the anastomosis. The right pulmonary artery was visualized via that anastomosis. Tuberculous pleurisy in the patient's history was considered to be the cause of the anastomosis. The therapeutic procedure used was embolization via a catheter for the anastomosis, but most anastomoses could not be embolized. The present report is considered the first on anastomosis of the intercostal artery to the pulmonary artery to the extent that high-output heart failure occurs.