Philippine Heart Center

Abstract Introduction Congenital lobar emphysema (CLE) is a rare developmental lung malformation characterized by overexpansion of a pulmonary lobe and compression of surrounding tissue normal tissue of the ipsilateral lung. Although 50% of cases present in the newborn period, diagnosis can be delayed when CLE coexists with congenital heart disease (CHD), as overlapping symptoms such as tachypnea and cyanosis are often attributed to cardiac dysfunction. Approximately 14-20% of patients with CLE have associated CHD, most commonly a ventricular septal defect (VSD). We report a case of an infant with Tetralogy of Fallot (TOF) and Absent Pulmonary Valve who developed unexpected postoperative respiratory distress and difficulty with ventilation, which uncovered a missed pulmonary malformation. Case Presentation A three-month-old girl with TOF and Absent Pulmonary Valve underwent corrective cardiac surgery. Preoperative chest radiograph showed right lung hyperaeration with mediastinal shift, but this finding was not initially recognized as CLE. Intraoperatively, the right lung appeared markedly emphysematous with leftward mediastinal deviation. Postoperatively, the infant developed persistent respiratory distress and difficulty in ventilation. Repeat chest imaging showed worsening left lung collapse, compensatory hyperinflation, and herniation of the right lung across the mediastinum. Despite intensive ventilatory support, she remained dependent on the ventilator. Persistent wheezing was managed with corticosteroids, magnesium sulfate, and nebulized bronchodilators. With gradual clinical improvement and resolution of infection, she was transitioned to non-invasive positive pressure ventilation, then to room air. She was discharged after 47 hospital days—43 days post-surgery and 19 days post-extubation. Discussion CLE is estimated to occur in 1 in 20,000-30,000 live births and is more common in males. It usually involves the left upper lobe but may affect any lobe. The condition results from partial bronchial obstruction due to intrinsic cartilage deficiency or external vascular compression, creating a “ball-valve” effect that traps air and causes lobar hyperinflation. In infants with CHD, CLE can remain silent until changes in pulmonary mechanics after surgery unmask the underlying defect. Conclusion CLE can present unexpectedly in the postoperative period. Although uncommon, it is an important differential diagnosis in infants presenting with respiratory distress particularly those with coexisting CHD. Preoperative imaging should be carefully reviewed to identify such pulmonary anomalies. Early recognition and appropriate multi-disciplinary team management are essential to prevent complex cardiopulmonary complications. This abstract is funded by: None

Hughes-Stovin syndrome (HSS) is a rare and aggressive type of systemic vasculitis which is characterized by peripheral venous thrombosis and pulmonary artery aneurysms (PAAs) at the onset of the disease, as well as the absence of recent or previous attacks of uveitis. The onset of HSS and its dominant clinical disease presentations differ in many aspects from those observed in Behçet’s disease (BD). The absence of uveitis is a mandatory exclusion criterion in the preliminary diagnostic criteria proposed by the HSS International Study Group (HSSISG), to avoid misclassification between HSS and BD. The presence of PAAs is an obligatory “entry criterion” for diagnosis, while vascular thrombotic events are a “major criterion” after excluding other causes of coagulopathy-induced thrombosis. The HSSISG regards HSS and BD as one disease, yet exhibiting distinct patterns of disease expression, particularly at disease onset. Differentiating HSS from BD is especially important for early diagnosis and optimal management. While the presence of HLA-B51 is the strongest genetic risk factor for BD, it is not used as a criterion to confirm a diagnosis because it lacks sufficient specificity. The prevalence of HLA-B51 in HSS is relatively low, suggesting that different genetic drivers might exist or that HSS is a unique entity with overlapping features with BD. In HSS, both HLA-B51 positive and negative cases have been described; thus, it cannot be a hallmark of the syndrome, and it does not play a role in the diagnostic criteria.

Abstract Introduction Lung adenocarcinoma may mimic infectious diseases as both entities may present with cough, dyspnea and other constitutional symptoms such as fever, weight loss and body malaise. Pneumonic-type lung adenocarcinoma, although rare, may appear as lobar consolidations on chest radiographs posing a diagnostic challenge. Case Report We present a case of a 57-year old Filipino male, previous smoker, who presented with 3-month history of recurrent productive cough associated with intermittent fever and weight loss. On initial consult, chest radiograph showed presence of consolidation-atelectasis on the right mid lung. Chest CT scan revealed lobar consolidation of the right middle lobe with multiple calcifications and heterogenous enhancement. Primary consideration was a large granuloma formation from pulmonary tuberculosis. Scattered few fibrotic densities and parenchymal nodules on both lungs were noted as well. Sputum TB Gene Xpert was negative for Mycobacterium tuberculosis DNA. Patient was managed as Clinically Diagnosed Pulmonary Tuberculosis and was started on Isoniazid, Rifampicin, Pyrazinamide and Ethambutol. However after completion of the 2-month intensive phase of anti-Koch’s regimen, cough and dyspnea persisted. There was no significant change on the lobar consolidation on Xray as well. Bronchoscopy with bronchial washing was then performed. Bacterial culture studies showed heavy growth of Acinetobacter baumanii and Klebsiella pneumoniae. Antibiotics were given which provided temporary relief. AFB smears, TB Gene Xpert, and KOH yielded negative results. CT-guided biopsy of the mass was also performed and histopathologic result showed cytomorphologic features consistent with primary lung adenocarcinoma with neuroendocrine features; proliferation index using KI-67 was <5%. Metastatic workups were done and oncology referral was facilitated. Discussion In the Philippines, lung cancer is the second most prevalent type of cancer in the country, but it remains as the leading cause of cancer-related death. Lung adenocarcinoma is a relatively common subtype however it may present with different clinical and radiographic features causing a diagnostic challenge especially in resource-limited settings. Due to the high TB burden in the country, patients presenting with granuloma formation and other radiologic findings suggestive of tuberculosis are clinically managed as such. However high index of suspicion must be given in cases unresponsive to medications as lung cancer may present with benign features such as consolidations or calcifications as well. Delay in diagnosis may lead to disease progression and poor outcomes. This abstract is funded by: None