Recurrent Episodes Of Dyspnea Research Articles

PS-R04-2: A CASE OF SUCCESSFUL PERCUTANEOUS TRANSLUMINAL RENAL ANGIOPLASTY (PTRA) FOR RENAL ARTERY STENOSIS ASSOCIATED WITH TAKAYASU'S ARTERITIS

Introduction: In Japan, renal vascular hypertension based on renal artery stenosis (RAS) accounts for 1% of all hypertensive patients, and RAS is present in approximately 10% of autopsy cases of coronary artery disease and cerebrovascular disease. As ischemic nephropathy and complications of cardiovascular disease (CVD) due to RAS are common, early diagnosis and treatment for RAS is important. However, results of several large clinical trials have not shown the efficacy of percutaneous transluminal renal angioplasty (PTRA) for atherosclerotic RAS. Case: 51-year-old woman who diagnosed as Takayasu's arteritis (TA) 35 years ago, had been treated with 5 mg daily of prednisone. She presented with hypertension and edema in both legs a year before admission. Her examination at that time revealed increased serum creatinine from 1.0 to 1.7 mg/dL, high proteinuria (7.3 g/gCr), high renin activity (47.0 ng/mL/hr) and right RAS. Eight months before admission, she had an operation for ruptured descending aorta. She had recurrent episodes of dyspnea on exertion, worsening leg edema and severe hypertension and was admitted to the hospital for the treatment. Clinical course: In addition to severe hypertension (190/80 mmHg) and progression of RAS, she had pulmonary congestion, high brain natriuretic peptide (BNP) level (1149.5 pg/mL), increasing creatinine levels (Cr 3.1 mg/dL), and left non-functioning kidney on admission. Despite taking 8 types of antihypertensive drugs and diuretics without renin-angiotensin system inhibitors, blood pressure and fluid retention were poorly controlled, and thus we decided to perform PTRA for the right RAS. After the PTRA, edema, hypertension and pleural effusion had been dramatically improved. In addition, we could observe decrease of serum creatinine (Cr 1.6 mg/dL), proteinuria (0.5 g/gCr) and plasma renin activity (1.9 ng/mL/hr). Therefore, we reduced the antihypertensive drugs from 8 to 2. Discussions: Although the benefit of PTRA for RAS associated with TA remains unclear, the clinical course suggests that the success of PTRA in this case may be related to the fact that the RAS was complicated by TA. It also suggests that we should consider PTRA in cases of hypertension and pulmonary congestion associated with RAS which are difficult to control like this case. Conclusion: We have experienced a case of RAS associated with TA that was successfully treated with PTRA.

Hypersensitivity pneumonitis in a teenager

Abstract Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD), consequence of an alveolar allergic reaction against various inhaled allergens occurring in susceptible individuals, manifesting as an acute or chronic granulomatous alveolar allergic process against inflammation of the lung parenchyma. The clinical presentation can mimic acute respiratory infections (in acute form) or an idiopathic ILD (in chronic form); the diagnosis of HP is difficult if the exposure to allergen is not suspected. We present the case of a male teenager, pigeon breeder, presenting with recurrent episodes of dyspnoea and fever, initially considered and treated as pneumonia. The diagnosis of HP was based on suggestive imaging changes, lymphocytic alveolitis at bronchoalveolar lavage with a low CD4/CD8 ratio and a thorough anamnesis for exposure and positive IgG serum precipitins against pigeon debris. The patient improved over a few months only by avoiding exposure to the incriminated allergen. ILDs in children and adolescents are considered rare diseases, with HP being one of the possible causes in older children and adolescents.

RECURRENT ARDS IN THE SETTING OF POLYSUBSTANCE ABUSE

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Drug abuse remains a significant social problem in many countries. Adverse pulmonary complications of substance abuse are well documented, including diffuse alveolar haemorrhage, aspiration pneumonitis, and opportunistic infections related to HIV and hepatitis. Acute lung injury and acute respiratory distress syndrome are well known complications of narcotic overdose, but ARDS from drug use without overdose is a much rarer entity. [1] CASE PRESENTATION: We report a case of a 39 year old female, current 10 pack-year smoker, with a history of polysubstance abuse including heroin, cocaine and alcohol, who was admitted 5 times in 8 months with recurrent episodes of dyspnea, fever, myalgias and fatigue. Each time she presented with hypoxic respiratory failure and bilateral infiltrates on chest imaging, and required high flow oxygen and BIPAP. She was treated with IV antibiotics, steroids and diuresis with good response. An extensive workup was performed including negative cultures, negative connective tissue disease serologies, and negative HIV testing. Echocardiogram showed a normal ejection fraction and negative bubble study. CT scan during the first hospitalization showed diffuse bilateral ground-glass opacities with bilateral lower lobe predominance and subpleural sparing without bronchiectasis or subpleural honeycombing. Outpatient PFTs showed only a moderately decreased diffusing capacity; methacholine challenge test showed mild bronchial hyperresponsiveness. Patient underwent bronchoscopy during one hospitalization that showed grossly normal airways, neutrophil predominant BAL and no eosinophilia; endobronchial biopsy showed atypical bronchial epithelium favoring reactive process. Surgical lung biopsy revealed rare intracapillary granulomas with exogenous polarizable material – microcrystalline cellulose consistent with IV drug injection. DISCUSSION: Pulmonary foreign body granulomatosis is described in patients who inject oral drugs intravenously. [2-3]. This would not account for the fulminant respiratory failure with ARDS in this patient. A case series of 6 patients using narcotics, gabapentin, and other psychoactive drugs, with presentation similar to our patient, with recurrent ARDS and diffuse alveolar damage on lung biopsy, has been reported [4]. DAD was not identified on this patient’s surgical lung biopsy, perhaps because the patient was biopsied in between recurrences. Management of this condition is unclear with some reports indicating a potential benefit from systemic glucocorticoids [5]. Some patients with recurrent ARDS progress to worsening fibrosis and may eventually need lung transplant. Our patient has been managed with recurrent prolonged prednisone taper, but further treatment is complicated by her social issues and medication noncompliance. CONCLUSIONS: DAD is pathologic manifestation of severe acute lung injury, recurrence can be attributed to polysubstance abuse. Reference #1: [1] Benson MK, Bentley AM. Lung disease induced by drug addiction. Thorax. 1995; 50(11): 1125–1127. Reference #2: [2]Bishay A, Amchentsev A, Saleh A, Patel N, Travis W, Raoof S. A hitherto unreported pulmonary complication in an IV heroin user. Chest 2008;133(2)549-51. Reference #3: [3] Radow SK, Nachamkin I, Morrow C, et al. Foreign body granulomatosis. Clinical and immunologic findings. Am Rev Respir Dis 1983;127:575. [4] Savici D, Katzenstein A-LA. Diffuse alveolar damage and recurrent respiratory failure: report of 6 cases. Human Pathology 2001;32(12):1398-402. [5] Chau CH, Yew WW, Lee J. Inhaled budesonide in the treatment of talc-induced pulmonary granulomatosis. Respiration. 2003;70(4):439. DISCLOSURES: No relevant relationships by Moayad Al Sona, source=Web Response No relevant relationships by Sandrine Hanna, source=Web Response No relevant relationships by Margaret Zambon, source=Web Response

Pseudotumor cerebri controlled with ventriculo-peritoneal shunt as a rare complication of ROHHAD syndrome: a case report

Background: rapid-onset obesity with hypoventilation, hypothalamic dysfunction and autonomic dysregulation (ROHHAD) is a rare yet possibly fatal condition. It is similar to common obesity syndromes (whether exogenous or genetic) sideways with other endocrinopathy in early childhood. Objective of the study: this study aimed to present the clinical manifestations, laboratory and imaging results as well as complications and treatment particularly for ventriculo-peritoneal shunt for a ROHHAD patient with pseudotumor cerebri. Patient and methods: in this case report, a 10-year-old girl was presented to the emergency department with recurrent episodes of dyspnea, hypernatremia and cyanosis. Results: her obesity was controlled with sleeve surgery and hormonal evaluation showed high prolactin level and hypothyroidism. She continued to have symptomatic hypoventilation and severe Pseudo-Tumor Cerebri (PTC), idiopathic intracranial hypertension (IIH), in addition to significant reduction in her visual acuity. She was diagnosed with ROHHAD syndrome based on the clinical, laboratory and radiological findings. Brain and abdominal magnetic resonance imaging were performed to demonstrate a possible accompanying neural crest tumor, which was normal, but follow-up images were recommended. Conclusion: ROHHAD syndrome should be suspected in all cases of rapid onset obesity associated with hypothalamic-pituitary endocrine dysfunction. Also provided the high prevalence of cardiorespiratory arrest and potentially associated neural crest tumors, early recognition of ROHHAD syndrome is quite crucial to avert mortality and morbidity. Moreover, Early ventriculoperitoneal shunt insertion might be a lifesaving procedure to avoid visual impairment and subsequent blindness in severe pseudotumor cerebri.

A case of recurrent and progressive respiratory failure

Introduction Patients presenting with dyspnea are common. Often times, patients carry previously anchored diagnoses, such as COPD, as a cause of their pulmonary symptoms. It is important, however, to perform a thorough history and physical examination in order to consider less common causes of dyspnea, such as in the case of this patient who was diagnosed with amyotrophic lateral sclerosis (ALS). The typical course for this disease process includes progressive limb and bulbar muscular weakness with eventual involvement of the respiratory musculature, ultimately leading to respiratory failure - the most frequent cause of death in ALS within 2 to 5 years of diagnosis. Case description A 68-year-old woman presented to the hospital with recurrent episodes of dyspnea and carbon dioxide retention. She has a history of type II diabetes, hyperlipidemia, hypertension, cervical stenosis, and chronic obstructive pulmonary disease (COPD) requiring home oxygen therapy. Her medications included the following COPD regimen: short acting anticholinergic/beta agonist inhaler, mucolytic, steroid, long acting beta agonist nebulizers, and a Trilogy adaptive servo-ventilation device for nighttime breathing assistance. In the ED, workup showed pH 7.34, pCO2 95mmHg, and HCO3 of 50mmHg; chest x-ray was significant for mild hyperexpansion. On examination, she had mild proximal upper extremity weakness, bilateral thenar atrophy, and a nasal voice. She was in the ICU for 24-48 hours for intensive positive pressure therapy because of her severe carbon dioxide retention. Results and conclusions Bedside pulmonary function testing was consistent with a restrictive process, and she was diagnosed with obesity hypoventilation. However, her BMI was only 39, and given her history of weakness and thenar atrophy, we were concerned for a neurologic process. Neurology found fibrillations with insertion and prominent fasciculations within the proximal right upper limb muscles on needle electromyography. Ultrasound examination with phrenic nerve stimulation showed reduced recruitment of large, complex motor unit potentials in both hemidiaphragms and intercostal muscles. With this constellation of symptoms, ALS was diagnosed. Other possible diagnoses were ruled out with neuroimaging, serologic, and cerebrospinal fluid studies. Take-home message Progressive dyspnea as the major presenting symptom of ALS is exceedingly rare, occurring in less than 1% according to literature. It is important to keep ALS in the differential diagnosis in patients who present with progressive dyspnea and restrictive lung disease on pulmonary function testing because this diagnosis has significant prognostic difference compared to other entities such as obesity hypoventilation syndrome.

Hyperventilation and seizures in an adolescent female

A 16 year-old girl was admitted after suffering from recurrent episodes of dyspnea and stridor, cyanosis, loss of contact, stiffening of all four limbs, clenching of the jaw and eye retroversion that lasted for a few seconds to a minute, followed by slow recovery of consciousness without any loss of sphincter control. These symptoms began at the age of 11 and worsened over time. Prolonged rate corrected QT intervals was observed with an ECG. Two cardio-respiratory monitorings were performed (one during daytime hours while the patient was awake, and one at night time while the patient slept). Daytime recordings showed 17 central apnoeas and 97 central hypopneas, with an apnea-hypopnea index (AHI) of 13.2 events/hour, that were associated with severe oxyhemoglobin desaturation. In contrast, night time recordings were normal (AHI=1.1 events/hour). The patient underwent diurnal monitoring of transcutaneous pCO2(PtcCO2), transcutaneous O2(PtcO2), SpO2 and end tidal CO2 (PETCO2), with simultaneous monitoring of regional cerebral oxymetry (rSO2) which showed values of PtcCO2 between 8 and 15 mmHg, suggesting several episodes of marked hyperventilation. Twenty-nine episodes of severe arterial desaturation (SpO2<50%) were registered, all after the same number of apnea events, with ascent of PtcO2 up to 28 mmHg at the end of apnea. During the final phase of apnea, the patient showed cyanosis, contact disturbance, grimaces, oral movements of rhyme, and, on three occasions, partial seizures. A mask was packaged and applied daytime to the face of the patient during episodes of hyperventilation to prevent, together with a psychiatric follow up, rapid falls of PaCO2 levels determining central apnoeas.

Management of Chronic Asthma in Adults

Asthma is a chronic inflammatory disease of the airways. It is characterized by recurrent episodes of dyspnoea, wheeze and cough caused by reversible airways obstruction. Three factors are responsible for the airways narrowing: bronchial muscle contraction, mucosal swelling/inflammation and increased mucous production ( Fig. 1 ). About 10–15% of the UK population have asthma and the number appears to be growing although there is some difficulty understanding why. It affects 1 in 20 adults and 1 in 10 children. Approximately 5.1 million people are currently being treated for asthma in the UK.

Methacholine Challenge Testing in the Diagnosis of Paradoxical Vocal Fold Motion

The purpose of this study was to determine whether methacholine challenge testing (MCT) combined with serial laryngoscopy could elicit and visualize paradoxical vocal fold motion (PVFM) during asymptomatic periods and suggest laryngeal dysfunction as an etiology of episodic dyspnea. A retrospective study of seven adult patients. Medical records of seven adult patients who were referred to an otolaryngology clinic with recurrent episodes of dyspnea or choking and underwent bronchoprovocation testing were reviewed. After initial fiberoptic laryngoscopy and pulmonary function testing (PFT), bronchoprovocation was performed using aerosolized methacholine at increasing concentrations. Each administration was followed by repeated laryngoscopy and PFT. A positive endoscopic test was marked by the visualization of paradoxical adduction of the true vocal folds during inspiration. A positive bronchoprovocation test was marked by a 20% or greater decline in the forced expiratory volume. All seven patients demonstrated normal upper airway anatomy and function on initial laryngoscopy. In three patients, a positive endoscopic examination demonstrating PVFM was elicited during MCT. In three, the bronchoprovocation test was positive, and asthma was diagnosed. In one patient, both tests were negative. PVFM was seen in all patients whose chief complaint was choking (n = 2) but in only one of five patients whose chief complaint was dyspnea. PVFM may be elicited and observed during MCT coupled with serial laryngoscopy. This test holds promise in differentiating vocal cord dysfunction from asthma during asymptomatic periods in patients who present with recurrent episodes of refractory dyspnea.

A rare but life-threatening complication of ventriculo-atrial shunt

Insertion of ventriculoperitoneal and ventriculoatrial shunts is routinely performed. Infarction pneumonia and atrial thrombus formation are described as very rare complications of ventriculoatrial shunts. We present the case of a female patient with ventriculoatrial shunt insertion as long term treatment for aequeductal stenosis who presented with recurrent episodes of dyspnoea, chest pain, and unilateral pleural effusion. Diagnostic evaluation revealed a positive D-dimer test, bilateral basal infiltrates and pleural effusion. Transesophageal echocardiography established the diagnosis of a thrombus in the right atrium. Laboratory testing for thrombophilia revealed a homozygous factor V Leiden mutation. In the following, a shunt revision was performed.

Non-invasive hemodynamic evaluation of patients with LV dysfunction and intractable dyspnea with first pass radionuclide angiography: comparison with echocardiography

Introduction: Advanced heart failure is characterized by recurrent episodes of dyspnea with or without obvious signs of congestion and fluid retention. These patients have many potential cardiac and pulmonary causes for recurrent dyspnea and repeating an echo each time is often uninformative and frequently limited technically in CHF patients, particularly if significant pulmonary disease also exists. We therefore sought to use first pass radionuclide angiography (FPRNA) to non-invasively evaluate the hemodynamic status of these very symptomatic and failing patients. Methods: Consecutive patients with LV dysfunction and severe dyspnea underwent upright FPRNA after injection of 15-45 millicurie of sestamibi. Imaging was performed using a Sim-400 multicrystal camera. All hemodynamic data were based on acquired raw and derived data. Included patients also underwent echocardiography which was independently reviewed. Results: 52 patients (80% male) met criteria. Mean age 68.7 years. Mean LVEF by echo 29.7% and 32.2% by FPRNA (NS p=0.53). Mean RVEF by FPRNA was 43.85%. 65% had RVEF <40%. Peak LV filling was 1.41 +/− 0.58 (normal value is >2.5), suggesting marked diastolic dysfunction. Mean cardiac output was 6.2 and mean index was 3.2. The LV was markedly enlarged in this population reflecting advanced heart disease. Mean LV end diastolic volume was 230 cc and end systolic volume was 162cc, both values over twice the accepted normal range. 69.2% had MR by echo (21.2% were rated as 1+, 13.5% as 2+, 17.3% each as 3+ and 4+). Most importantly, the normalized mean pulmonary transit time (corrected for heart rate) was found to directly correlate with the degree of MR on echo (Spearman's rho 0.346, p=0.02). This has not been previously shown in severe CHF. Conclusion: In this study we sought to establish that reliable non-invasive hemodynamic data can easily be obtained from very symptomatic patients with LV dysfunction. The advantages of FPRNA are that patients are imaged upright and the results are quantitative. Accurate evaluation of MR is possible by normalizing the PMTT, thereby allowing this normally subjective echo data to be quantified and followed serially. In addition quantitative data on LV and RV systolic function, LV diastolic function and pulmonary circulation can be obtained and followed serially. By comparing such serial results, abnormalities in specific parameters may yield clues to whether dyspnea is of cardiac or pulmonary origin in severe CHF.

Hemangioma of the tricuspid valve: A report of two cases with review of literature

We describe two cases of hemangioma affecting the tricuspid valve (TV). One patient was a 47-year-old male who presented with recurrent episodes of dyspnea on exertion for 5 years. Echocardiography revealed an echo dense 1×1 cm mass attached to the chordae tendineae of the TV. Surgical excision was done and histopathology of the lesion showed features of hemangioma. The other case was a 1-day-old neonate with complex congenital abnormalities in whom multiple small hemangiomas affecting the TV were incidentally detected at autopsy.

30-Year-Old Man With Recurrent Episodes of Dyspnea

30-Year-Old Man With Recurrent Episodes of Dyspnea

Pulmonary Manifestations in Behçet's Syndrome

Among 72 patients with Behçet's syndrome, seven had pulmonary vascular involvement. Additional data from 42 cases in the literature are discussed. Recurrent episodes of dyspnea, cough, chest pain, and hemoptysis were the primary clinical signs, mainly in young men, appearing 3.6 years after the first manifestation of Behçet's syndrome. Fever, elevated ESR, and anemia were common, and chest x-ray films showed pulmonary infiltrates, pleural effusions, and prominent pulmonary arteries. Ventilation-perfusion scans showed perfusion defects even when chest x-ray films were normal. Pulmonary artery aneurysms were seen in 7/13 in whom angiography was done. Of 42 patients, 16 died, 15 from fatal pulmonary hemorrhage, 80 percent within two years from the development of pulmonary disease. Histopathologic study results showed vasculitis of pulmonary vessels of various sizes, leading to thrombosis, destruction of the elastic laminae, aneurysms, and arteriobronchial fistula. In addition, pulmonary emboli and the aphthous lesion of the tracheobronchial tree may aid the clinical picture. Anticoagulant therapy may be hazardous in patients with aneurysmal dilatation of the pulmonary vascular tree, and the beneficial effect of corticosteroid therapy is discussed. Pulmonary vasculitis in Behçet's syndrome is a unique clinical and pathologic picture, differing from other vasculitides affecting the lung, presents a major threat to the patient's life.