Recurrent Haemoptysis Revealing Hughes-Stovin Syndrome

Hughes-Stovin syndrome is a very rare condition with no defined diagnostic criteria. We present the case of a 26-year-old man who had haemoptysis revealing Hughes-Stovin syndrome. We will consider the aetiology, therapeutic and evolutionary aspects of this disease.

Recurrent Haemoptysis Revealing Hughes-Stovin Syndrome

Hughes-Stovin syndrome is a very rare condition with no defined diagnostic criteria. We present the case of a 26-year-old man who had haemoptysis revealing Hughes-Stovin syndrome. We will consider the aetiology, therapeutic and evolutionary aspects of this disease.

Case report of recurrent haemoptysis in an older patient with repaired tetralogy of Fallot

Background Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Although most infants nowadays undergo surgical repair approximately at the age of 6 months with excellent outcomes, older patients typically underwent a staged approach with an initial systemic-to-pulmonary Blalock–Taussig–Thomas (BTT) shunt reducing hypoxaemia, followed by surgical TOF repair at an older age (with takedown of the BTT shunt). Late complications related to the BTT shunt are rare. Case summary We report a case of recurrent haemoptysis late after classic BTT shunt and subsequent surgical TOF repair. Axial imaging indicated a stellate nodule with isotope accumulation in the right upper lung lobe, whereas bronchoscopy showed a rope-like structure in the bronchus to the right upper lung lobe. The patient underwent a lobectomy of the superior right lobe, with identification of what appeared to be the old suture around the BTT shunt. Anatomopathology confirmed diffuse necrotizing inflammation with erosion into the bronchus which eventually caused haemoptysis. Discussion Management of older patients with congenital heart disease requires a thorough knowledge of the anatomy and surgical history of the patient. Although late complications related to a BTT shunt are rare, a residual shunt, endocarditis, pseudoaneurysm, or chronic inflammation with haemoptysis may occur.

Long-Term Outcomes of Bronchial Artery Embolization for Patients with Non-Mycobacterial Non-Fungal Infection Bronchiectasis

<b><i>Background:</i></b> There is no study on the predictive factors of recurrent haemoptysis after bronchial artery embolization (BAE) with the long-term outcomes in patients with bronchiectasis (BE). <b><i>Objectives:</i></b> To evaluate the long-term outcomes of BAE in BE patients without accompanying refractory active infection of mycobacteriosis and aspergillosis with analysis for the predictive factors of recurrent haemoptysis. <b><i>Methods:</i></b> Data of 106 patients with BE who underwent BAE using coils between January 2011 and December 2018 were retrospectively reviewed. The cumulative haemoptysis control rate was estimated using Kaplan-Meier methods with log-rank tests to analyze differences in recurrence-free rate between groups based on technical success and failure, bacterial colonization status, number of BE lesions, and vessels embolized to bronchial arteries (BAs) or BAs + non-bronchial systemic arteries (NBSAs). <b><i>Results:</i></b> Bacterial colonization was detected in approximately 60% of patients. Computed tomography showed bronchiectatic lesions with 2.9 ± 1.4 lobes. In the first series of BAE, embolization was performed in the BAs alone and BAs + NBSAs in 65.1 and 34.9% of patients, respectively, with 2.4 ± 1.4 embolized vessels in total. The median follow-up period was 1,000 (7–2,790) days. The cumulative haemoptysis control rates were 91.3, 84.2, 81.5, and 78.9% at 1, 2, 3, and 5 years, respectively. The haemoptysis control rates were higher in the technical success group than in the technical failure group (<i>p</i> = 0.029). <b><i>Conclusions:</i></b> High haemoptysis control rates for long-term periods were obtained by embolization for all visualized abnormal arteries, regardless of the colonization status, number of bronchiectatic lobes, and target vessels, irrespective of NBSAs.

Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

Unilateral pulmonary vein atresia presenting with recurrent haemoptysis in a child: a case report

Background Haemoptysis is an uncommon presenting symptom in children and is usually caused by acute lower respiratory tract infection or foreign body aspiration. We report a rare case of right unilateral pulmonary vein atresia (PVA) as the underlying aetiology of recurrent haemoptysis in a child. Case presentation A 4 years old girl presented with history of recurrent haemoptysis. Bronchoscopic evaluation excluded a foreign body aspiration but revealed right bronchial mucosal hyperaemia and varices. Diagnosis of right unilateral PVA was suspected on transthoracic echocardiography which demonstrated hypoplastic right pulmonary artery and non-visualization of right pulmonary veins. Final diagnosis was confirmed on cardiac CT angiography. A conservative treatment approach was opted with consideration for pneumonectomy in future when she is older. Conclusion Rarer causes should be considered when investigating for recurrent haemoptysis in children. Bronchoscopy and cardiac imaging are useful tools to establish the diagnosis of unilateral PVA in our case.

Monoparesis Post Bronchial Artery Embolization: Clinicoradiological Discordance in Anterior Spinal Artery Infarct—Case Report and Review of Literature

Bronchial artery embolization (BAE) is an established minimally invasive treatment for massive or recurrent haemoptysis. Anterior spinal artery (ASA) infarct is an uncommon but serious complication of the procedure. The interventional radiologist doing these procedures must be well versed with the bronchial artery anatomy, its variations, and its relation with ASA to avoid this dreaded complication. The authors report a case in which the patient developed monoparesis and mild transient autonomic dysfunction post BAE but magnetic resonance imaging of spine showed diffuse symmetric cord involvement in ASA territory from D1–D4 level.