Unilateral pulmonary vein stenosis.

Unilateral pulmonary vein stenosis is a rare congenital anomaly. A case is described in a girl who first presented at the age of four years with recurring haemoptysis but in whom diagnosis was not established until she was 16 years old. Pulmonary angiography demonstrated a minimally hypoplastic right pulmonary artery, and the laevophase showed normal pulmonary venous return from the left lung, but none from the right. Surgical treatment was necessary because of life threatening haemoptysis, and pneumonectomy was required in the light of the findings at operation.     

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.     

Pulmonary Hypertension in Takayasu's Arteritis

Summary: A 25 year old Chinese male presented with recurrent attacks of haemoptysis since the age of 14 years. He had a continuous murmur over the right upper chest and signs of pulmonary hypertension which was confirmed at catheterization. Pulmonary angiography and aortagram showed arteritis of the pulmonary vessels and the aorta.
Pulmonary involvement in Takayasu's arteritis does occur but is very rare. It is exceptional for a patient to develop pulmonary hypertension and present initially as a case of pulmonary vessels stenosis and occlusion. We describe one such case.     

A case report of congenital isolated absence of the right pulmonary artery: bronchofibrescopic findings and chest radiological tracings over 9 years

A 27-year-old man was admitted to hospital for investigation of haemoptysis. He was a serving member of the Japan Self-Defense Forces. A CXR showed absence of the right hilum, and the right hemithorax was smaller than the contralateral hemithorax. Pulmonary arteriography demonstrated complete absence of the right pulmonary artery. Right heart catheterisation did not show any concomitant cardiovascular malformations, and the patient was diagnosed as having isolated absence of the right pulmonary artery. Bronchial arteriograms demonstrated enlargement and proliferation of the small branches of the right bronchial artery. Bronchofibrescopic examination showed obvious enlargement of the bronchial mucosal vessels with submucosal eruptions and swelling in the right bronchial tree, but not on the left side. Re-examination of the patient's CXR from the previous 9 years revealed a chronological decrease of right lung volume and an increase of the cardiothoracic ratio. Isolated absence of the right pulmonary artery is generally considered to have a good prognosis, but close observation is necessary to monitor the pulmonary haemodynamics.     

Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger's syndrome: a clinical dilemma

Although the frequency of haemoptysis in Eisenmenger's syndrome is well recognised, the high prevalence of pulmonary artery thrombus has been newly appreciated through the growing use of non-invasive imaging. Three patients with Eisenmenger's syndrome with haemoptysis are reported who underwent computed tomography pulmonary angiography and cardiovascular magnetic resonance. Each patient was found to have aneurysmal dilatation of the right pulmonary artery with large laminar thrombus. These cases illustrate a rising clinical problem in this special population-that is, how to treat and prevent large pulmonary artery thrombosis in the setting of haemoptysis. The authors discuss their approach to these cases and the known literature.     

Coccidioidomycosis mimicking lung cancer

A 65-year-old female smoker was admitted due to haemoptysis and a pulmonary infiltrate in the right lower lung lobe. Tumour was suspected, but there was also a history of 2 years spent in Arizona, known to be endemic for the dimorphic fungus Coccidioides immitis. IgG antibodies and airway culture for C. immitis were negative, and surgery was performed for suspected lung cancer. No malignancy was detected at histopathological investigation, but typical coccidioidal spherulae were observed by silver stain. Coccidioidoma may be a differential diagnosis to malignant tumour in individuals visiting endemic areas.     

Subacute massive pulmonary embolism.

Twenty-four patients with subacute massive pulmonary embolism were studied both during their initial illness and up to nine years after it. The most common mode of presentation was progressive dyspnoea over a two to 12 week period, which in some, but not all, patients was accompanied by pleuritic chest pain and haemoptysis. Physical signs at diagnosis usually suggested right heart strain and ventilation/perfusion mismatch and in the five patients with the highest pulmonary artery pressures the pulmonary component of the second sound was accentuated. The chest x-ray and electrocardiogram provided useful diagnostic information in most patients though occasionally they were normal. Early response to thrombolytic treatment was poor when compared with patients with acute pulmonary embolism but was occasionally dramatically successful, and heparin alone provided satisfactory treatment in the eight patients receiving it. Pulmonary embolectomy provided poor results and four of the five patients undergoing this form of treatment died. Nine patients died during the initial illness and in seven death was directly related to embolic disease. One patient died from neoplastic disease during follow-up. Though the prolonged illness, poor initial response to treatment, and absence of predisposing factors suggest that recurrent embolic disease and late pulmonary hypertension might occur three was no evidence of this during a follow-up period of one to nine years (median five years).     

Embolization of a pulmonary arterial pseudoaneurysm with endovascular coils

Unrecognized pseudoaneurysm or rupture of a pulmonary artery is a rare but potentially catastrophic complication of pulmonary arterial catheterization. Herein, we describe a teenage patient with a pulmonary arterial pseudoaneurysm, probably iatrogenic, who presented with haemoptysis following catheterization of the right heart. The pseudoaneurysm was successfully embolized using coils inserted by catheter. Increased awareness of this lesion, its rapid recognition, and prompt therapy are the keys to a successful outcome.     

Unilateral pulmonary artery agenesia complicated by massive haemoptysis in an adult female

Isolated unilateral pulmonary artery agenesis is a rare congenital abnormality. We report a case of right pulmonary artery agenesis in an adult female without other cardiovascular anomalies. The patient presented with massive haemoptysis four years after the original diagnosis. The patient underwent right pneumonectomy with stapled suture of the main bronchus covered by an intercostal muscle flap. The clinical course was complicated by a bronchopleural fistula one month after the first operation. The fistula was successfully treated with a videothoracoscopic omentoplasty and serratus muscle flap. In these patients the surgical approach can be particularly complex because of the high risk of bleeding from the highly vascularised and extensive adhesions between the lung and the chest wall, associated with hyperplasia of the bronchial and intercostal arterial trees. In spite of these difficulties, access to the pulmonary veins and the main bronchus during pneumonectomy is not challenging.     

Pulmonary fibrosis predating microscopic polyangiitis by seven years

A 63-year-old man, ex-smoker with renal failure of recent onset was admitted at the respiratory department with massive haemoptysis. Previous X-rays and CT scans showed pulmonary fibrosis of seven-year duration. Subsequently, he developed high fever, large haemoptysis, new infiltrates and respiratory failure despite broad-spectrum antibiotic treatment. Antineutrophilic antibodies of the perinuclear type with specificity against myeloperoxidase were detected and microscopic polyangiitis was diagnosed. Immunosuppressive treatment with methylprednisolone pulses and cyclophosphamide was started with initially favorable response, but later the patient developed a hospital-acquired pneumonia which was treated successfully with meropenem. As pulmonary haemorrhage recurred, he was transferred to intensive care for plasmapheresis which was considered the last treatment option. Unfortunately he died from septic shock.ConclusionAsymptomatic pulmonary fibrosis can predate microscopic polyangiitis by several years and is associated with unfavorable prognosis of the vasculitis. Appreciation of this finding would lead to faster diagnosis and better management of these patients.     

Asymptomatic metalloptysis complicating lobectomy in pulmonary aspergilloma – An unusual complication

Recurrent haemoptysis in pulmonary aspergilloma is an indication for surgical resection of the cavity and removal of the fungus ball, in patients with a sufficient pulmonary function to allow surgery. Use of titanium surgical clips in such cardiothoracic surgical procedures may be the source of unusual complications. We report one such unusual complication of expectoration of a titanium surgical clip through a right lobectomy stump, the procedure having been performed six years previously for a symptomatic aspergilloma. We believe this to be first instance of metalloptysis in a case of pulmonary aspergilloma, following lobectomy.     

Outcome of patients with Eisenmenger syndrome. Apropos of 62 cases followed-up for an average of 16 years]

In an era when heart-lung transplantation offers a therapeutic option for patients with Eisenmenger's syndrome, it is important to assess the natural history of this condition. With this objective the authors studied 62 patients followed-up by the same cardiologist. The average follow-up period was 16 years, but 22 patients were followed up for over 20 years. The average age at death was 29 years. It differed significantly for genetically normal patients (31 years for 21 fatalities) compared with a population of trisomics (21 years for 6 fatalities). Half the patient population lived for over 30 years. Fourteen of the 27 deaths occurred during the third decade and only 4 before the age of 20. The probability of surviving 10 more years for a 20 years old genetically normal patient was 56%. The causes of death in the 19 cases in which it could be established were: 5 sudden deaths, 4 right heart failures, 3 massive haemoptyses, 3 pulmonary emboli, 2 pneumonias and 2 peroperative deaths. The functional disability was nearly always minimal or mild, enabling the patient to work: 24 of the 45 non-trisomic patients had full-time jobs. Pregnancy was a poor prognosis factor and could be lethal (2 deaths due to pulmonary embolism in the post-partum period). A heart-lung transplantation would only seem to be justified in patients with severe symptoms, polycythaemia, irreversible right heart failure and/or haemoptysis.     

Haemoptysis: aetiology, evaluation and outcome--a prospective study in a third-world country.

Haemoptysis is an alarming symptom, and the management depends upon the aetiology. Emergency management depends upon localization of the site of bleeding by roentgenogram, computerized chest tompgraphy and bronchoscopy. We prospectively evaluated 52 patients with haemoptysis admitted to the Chest Hospital, Kuwait for 1 year (January 1998 to December 1998) and followed them up for 1 year (January 1999 to December 1999). There were 42 males (80.8%) and 10 (19.2%) females, with a mean age of 42.2 (16-86) years. Of these, 26.9% were Kuwaiti nationals, 36.5% were Arab non-Kuwaiti nationals, 34.6% were Asians and 1.9% were other nationals. The aetiologies of haemoptysis were bronchiectasis (21.2%), old pulmonary tuberculosis with bronchiectasis (17.3%), active pulmonary tuberculosis (15.4%), bronchitis (5.8%), aspergilloma, rheumatic heart disease and carcinoid (1.9%). Aetiology could not be identified in 25% of patients. The site of bleeding in haemoptysis could not be localized by the consultants in 18 (32%) by roentgenogram. 16 patients (37%) by CT scan and 23 patients (50%) by Fibreoptic bronchoscopy. Sequential estimation of hemoglobin showed a mean of 13.56 (SD 1.9) and 13.31 (SD 1.8) after 24 h. The difference in mean was statistically significant (p<0.036). Conservative management was given in 80.8%, and embolotherapy or surgical intervention in 19.2% of patients. Only 12% of patients had recurrent haemoptysis at 1-year follow up. In conclusion, bronchiectasis and pulmonary tuberculosis were the major causes of haemoptysis in this study. Roentgenogram, CT scan and fibreoptic bronchoscopy are useful for localizing the site of bleeding. Sequential estimation of haemoglobin may be helpful in assessing the severity of haemoptysis, but larger studies are required to address this observation. The outcome of haemoptysis is generally good, with a low mortality and recurrence rate.     

Embolisation of hydatid cysts in the pulmonary artery presenting with haemoptysis

A 49-year-old female patient who had undergone surgery for hepatic echinococcosis five years previously was admitted with haemoptysis. MRI angiography showed total occlusion of the left inferior pulmonary artery. Echocardiography showed no pulmonary hypertension. The patient underwent pneumonectomy and cysts in the left pulmonary artery were observed. Pulmonary artery involvement should be considered in patients who have undergone hepatic cyst surgery if haemoptysis is the first presenting symptom, especially in endemic regions for hydatidosis.     

Unilateral absence of a pulmonary artery: congenital disease or embolic occlusion?

A young man with congenital absence of the left pulmonary artery is reported. A right aortic arch and normal pulmonary pressures essentially rule out an alternate diagnosis of occlusive chronic thrombus in the central pulmonary arteries. Recurrent or massive haemoptysis makes an exact knowledge of the pulmonary circulation advisable.     

Pulmonary mycotic aneurysms secondary to infective endocarditis in a patient with a persistent ductus arteriosus and partial anomalous pulmonary venous drainage

Mycotic aneurysms are a rare but recognized complication ofinfective endocarditis. Aneuryms of the pulmonary artery areusually associated with infected congenital heart lesions andespecially with persistent ductus arteriosus. This report dealswith a patient with a persistent ductus who developed multiplemycotic aneurysms in the right lung following infective endocarditis,and whose management was complicated by anomalous venous drainageof most of the contralacteral lung. Surgical closure of theductus to reduce pulmonary blood flow failed to prevent haemoptysis,and further surgery to ligate the feeder arteries to the aneurysmswas required.     

Occurrence of pulmonary artery aneurysms and pulmonary artery thrombosis in a young man

Beh?et's disease (BD) is a multisystemic inflammatory disorder of unknown aetiology. Arterial involvement is uncommon but associated with important morbidity and mortality. We describe the clinical course of BD with severe pulmonary artery involvement in a 19-year-old man. He presented with massive haemoptysis related to pulmonary artery aneurysms. Initial treatment consisted in urgent right inferior lobectomy, corticosteroids and monthly intravenous cyclophosphamide. Subsequently, he developed pulmonary artery thrombosis at non-aneurysmatic sites. Corticosteroid therapy was intensified, monthly intravenous cyclophosphamide was continued and an anticoagulant was added to the treatment with a favourable clinical and radiological response. Our case illustrates that timely initiation of highly potent immunosuppressive therapy is critical to obtain a favourable outcome. At present, a consensus regarding optimal management of vascular BD is lacking. ALthough anticoagulation is not generally recommended, our report encourages a patient-based decision after carefully tailoring potential risks and benefits.     

Tuberculosis of the Lower Lung Field

Summary: Pulmonary tuberculosis in adults is typically localised in the apices of the lungs. Lower lung field tuberculosis, although uncommon, is a well recognised entity which still occurs in countries with low or high prevalence of tuberculosis. Six patients with lower lung field tuberculosis, seen at the University of Papua New Guinea hospital over a period of one year, are described in this report. All six were Melanesian with a mean age of 32 years. Five were female. Fever, productive cough, pleuritic chest pain and localised crepitant rales over the affected area of the lungs were the most common clinical findings. Duration of symptoms prior to hospitalisation ranged from two to 12 months (mean: eight months). Four patients had haemoptysis. Right lung was affected more often than the left. The diagnosis of tuberculosis was delayed in four patients owing to the atypical localisation of the pulmonary infiltrates and to the absence of acid fast bacilli in the first three sputum smears. Lower lung field tuberculosis occurs more commonly in young females, affects the right lung more often and is associated with haemoptysis, early cavitation and hilar lymphadenopathy. Atypical location of the infiltrate may result in mis-diagnosis as lobar pneumonia, lung abscess or carcinoma of the lung.     

Intracardiac thrombus and pulmonary artery aneurysm in Behcet's disease

Intracardiac thrombus is a rare manifestation of Behcet's disease. OBSERVATION: we report the case of a 60 year old male patient with Beh?et's disease who presented with dyspnea and haemoptysis. CT angiography and echocardiography was performed allowing the identification of right heart thrombus associated with pulmonary artery aneurysm. The outcome was favourable with medical management. CONCLUSION: Intracardiac thrombus is a rare complication of Behcet's disease, but the diagnosis should be sought early in order to allow medical management to be considered.