ABSTRACT
Background: Diffuse parenchymal lung diseases (DPLDs) have gone through various changes in nomenclature and classification since they were first described in 1868. Increasing knowledge about their etiopathogenesis has since led to several reclassifications and changes in the nomenclature. This has had a major impact on the prevalence of each interstitial lung disease (ILD) reported by the different registries worldwide. In this study, we attempted to describe the distribution of the different DPLDs in our population and reported changes in prevalence due to changing diagnostic criteria for the disease. Materials and methods: We analyzed retrospective data of 434 patients. For the initial 75 patients, ATS/ERS guidelines published in 2002 were followed in the diagnosis of the ILD (group I). In the later part of the study (359 patients), the diagnosis was based on the computed tomography (CT) patterns defined by ATS/ERS/JPS/ALAT statement on diagnosis of idiopathic pulmonary fibrosis (IPF) and updated 2013 ATS/ERS guidelines (group II). Results: Of the 75 patients in group I, IPF was the most common diagnosis (52%) made at that time, followed by sarcoidosis and connective tissue-related ILD (CTD-ILD) with 12% each. Group II had 359 patients, with IPF again being the most commonly diagnosed ILD with 21.3%. This was followed by CTD-ILD (18.6%), sarcoid (14.7%), and idiopathic nonspecific interstitial pneumonitis (iNSIP; 13.3%). The changing guidelines have an impact on reporting of different DPLD by our multidisciplinary teamover a period of time. Though IPF was the most commonest DPLD reported among both the groups, the diagnosis of IPF had fallen by more than half in the second group. It was paralleled by an increase in the diagnosis of iNSIP and chronic hypersensitivity pneumonitis. These reported changes in the prevalence of DPLDs may reflect the better-defined criteria in the latest guidelines and a better understanding of the fibrotic ILDs other than IPF by the multidisciplinary team. Conclusions: The frequency of diagnosis of the different DPLDs has changed, following the publication of several guidelines in the last decade. It has recognized newer entities with greater clarity, such as idiopathic NSIP and interstitial pneumonia with autoimmune features.
ABSTRACT
BACKGROUND: Modified Blalock-Taussig shunts are used to palliate a variety of cyanotic heart diseases associated with reduced pulmonary blood flow. Acute shunt thrombosis in patients with shunt-dependent pulmonary blood flow can result in life-threatening hypoxia. We describe our experience of emergency transcatheter recanalization in 5 severely hypoxic children with acute shunt occlusion. METHODS AND RESULTS: Five patients with ages ranging between 5 and 24 months (median 11 months) and weight ranging from 4 to 8 kg (median 5 kg) presented with severe hypoxia, acidosis and hypotension following acute occlusion of modified Blalock-Taussig shunts placed 11 days to 12 months ago. As severe hypoxia (saturation range 3 5%-5 0%), acidosis and a state of shock in all the patients increased the risk for a redo surgical procedure, they were taken up for emergency transcatheter recanalization within 2-6 hours of hospitalization. This was done by positioning a Judkin's right coronary catheter at the mouth of the thrombosed shunt, crossing the shunt with a guidewire and serial dilatations with coronary and/or peripheral angioplasty balloons to the size of the graft. This technique was immediately successful in 4 of the 5 patients, thereby avoiding a repeat palliative operation. In 2 patients with residual stenosis, stents were used to restore luminal patency. One patient with acute stent thrombosis was managed successfully with local delivery of thrombolysis for 36 hours, which resulted in good luminal patency. At follow-up after 6 and 12 months, the shunts in both the stented patients are patent, with an oxygen saturation of 78% and 80%, respectively. In 2 other patients who had undergone plain balloon angioplasty, the shunts remained patent for 11 days (died of bronchopneumonia and septicemia) and 3 months, respectively. The procedure was unsuccessful in one very sick patient in whom the shunt had a tortuous course. CONCLUSIONS: Transcatheter recanalization of an acutely thrombosed Blalock-Taussig shunt is feasible. It can offer satisfactory short-term palliation in selected patients. Stents may play a role in patients with residual narrowing after dilatation. The procedure can be expeditiously accomplished in an emergency situation in a severely hypoxic child and may be a realistic alternative to surgery or thrombolysis.