Platypnea-Orthodeoxia Syndrome: A Case of Chronic Paroxysmal Hypoxemia.

A 75-year-old man with chronic (30-year) unexplained paroxysmal hypoxemia presented with postural hypoxemia and desaturation consistent with a clinical manifestation of platypnea-orthodeoxia syndrome. His history included a lack of significant past pulmonary disease, yet with intermittent need for oxygen supplementation. On admission he was found to have an interatrial shunt through a patent foramen ovale. Device closure by percutaneous catheterization led to sustained resolution of symptoms. Platypnea-orthodeoxia syndrome is a rare but important consideration in the differential diagnosis of hypoxemia, as it represents a potentially curable cause of hypoxemia, with missed diagnosis leading to possible patient morbidity if untreated. Even more importantly, an astute and careful history and physical examination are integral to the diagnosis of this rare but likely under-recognized syndrome.

Prevalence of Febrile Seizures, Epilepsy, and Other Paroxysmal Attacks in a Swedish Cohort of 4-Year-Old Children.

A questionnaire about any type of seizures was distributed to parents at the children's 4-year health surveillance at Child Healthcare Centers in Gothenburg, Sweden, to analyze the prevalence of febrile seizures (FS), epilepsy, and other paroxysmal attacks. Parents who reported any kind of seizures in their child were subsequently contacted by telephone to confirm the information given and to invite the child to a clinical assessment. In addition, hospital registers and individual records were checked of the appropriate age group as regards a diagnosis of epilepsy or febrile seizures. Parents of 4,290 of 6,076 eligible children (71%) completed the questionnaire. For 252 children (5.9%), any type of paroxysmal attack was reported: FS in 157/4,290 children (3.7%), epilepsy in 22/4,290 (0.5%), and other paroxysmal attacks in 75/4,290 (1.7%). Epilepsy developed in 4 out of 157 (2.5%) children with FS before their fifth birthday. This population-based study, covering all types of paroxysmal attacks in preschool children revealed a total prevalence of nearly 6%, the largest group being FS. The total rate of paroxysmal attacks in preschool children is equal to the rate of developmental/neuropsychiatric disorders in this age group. The conditions constitute a large group in pediatrics and entail considerable concern among parents.

ECG of the month. Cardiac failure and stroke in a 43-year-old woman. Coarse atrial fibrillation indicating left atrial enlargement and left ventricular hypertrophy with repolarization abnormality.

A 43-year-old woman with a long history of heavy cigarette smoking was in good health until she developed fatigue, dyspnea on exertion, and paroxysmal nocturnal dyspnea approximately three months before admission to our hospital. Four weeks before admission, she was admitted to another hospital for the sudden onset of a right hemiparesis. She was noted to be in atrial fibrillation, and cardiac catheterization and angiocardiography revealed triple-vessel coronary arterial disease and moderately severe mitral regurgitation. Because of repeated episodes of paroxysmal nocturnal dyspnea, she was referred to our hospital for cardiac surgery. On admission, an electrocardiogram was recorded (Figure).

Can patent foramen ovale affect rehabilitation? The uncommon association of platypnea-orthodeoxia syndrome and stroke.

Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea and deoxygenation induced by a change to a sitting or standing from a recumbent position. It is the result of posturally accentuated intracardiac or pulmonary right-to-left shunt leading to arterial oxygen desaturation. Only few cases of platypnea-orthodeoxia syndrome are reported in the literature and the association between stroke and platypnea-orthodeoxia syndrome with evidence of patent foramen ovale is extremely rare. We describe the case of a 67-year-old female admitted to our Rehabilitation Unit for disabling basilar stroke due to paradoxical embolism from patent foramen ovale that during the first days of rehabilitation showed signs and symptoms of platypnea-orthodeoxia syndrome. To remove a life-threatening condition for the patient and in order to develop the normal rehabilitation project, that was stopped by the platypnea-orthodeoxia syndrome, the patient fastly underwent to percutaneous closure of patent foramen ovale. The stabilization of oxygen arterial saturation with postural changes and the disappearance of symptoms of POS allowed to develop the rehabilitation project with progressive neurological improvement.

Contralateral diaphragmatic palsy after subcortical middle cerebral artery infarction without capsular involvement.

Diaphragmatic palsy after acute stroke is a novel clinical entity and may result in a high incidence of respiratory dysfunction and pneumonia, which especially cause greater morbidity and mortality. Generally, internal capsule and complete middle cerebral artery (MCA) infarctions are major risk-factors for developing diaphragmatic palsy. Herein, we present a case with contralateral diaphragmatic palsy after a subcortical MCA infarction without capsular involvement. Dyspnea occurred after stroke, while a chest X-ray and CT study disclosed an elevated right hemidiaphragm without significant infiltration or patch of pneumonia. A phrenic nerve conduction study showed bilateral mild prolonged onset-latency without any significant right-left difference. This suggested a lesion causing diaphragmatic palsy was not in the phrenic nerve itself, but could possibly originate from an above central location (subcortical MCA infarction). We also discussed the role of transcranial magnetic stimulation study in the survey of central pathway and demonstrated diaphragmatic palsy-related orthopnea.

Dyspnoeic dysphasia: a series of unfortunate events.

We describe a case of a 56 year old man with no previous medical history who presented with sudden onset dyspnoea, expressive dysphasia, and right arm sensory loss and paresis. A diagnosis of bilateral pulmonary embolism and transient cerebral ischaemic attack was confirmed by CT pulmonary angiogram and MRI. Paradoxical embolism through an occult patent foramen ovale (PFO) was subsequently proven by contrast echocardiography. This case highlights a number of short and long-term management conundrums, that to date are incompletely addressed by clinical trials. These include timing of anticoagulation in patients with both venous thromboembolism and cerebral infarction, and the risk:benefit ratio of surgical closure of patent foramen ovale.

Paroxysmal dyspnea in Parkinson's disease: Respiratory dyskinesias and autonomic hyperventilation are not the same.

Respiratory complaints are not uncommon in patients with Parkinson's disease (PD). While many are explained by pulmonary and cardiovascular problems unrelated to PD, secondary effects of PD, such as kyphoscoliosis, respiratory muscle rigidity, repeated pneumonias, or side effects of medication such as dyskinesias, there is a small group of patients with paroxysmal dyspnea for whom neither anxiety or other explanation has been found. This Point of View was written to call attention to this neglected, uncommon, but very distressing symptom.

Alveolar membrane conductance decreases as BNP increases during exercise in heart failure. Rationale for BNP in the evaluation of dyspnea.

BACKGROUND: In left ventricular failure (LVF) patients, brain natriuretic peptide (BNP), lung diffusion for carbon monoxide (DLCO), and alveolar-membrane conductance (DM) correlate with LVF severity and prognosis. The reduction of DLCO and DM during exercise reflects pulmonary edema formation. METHODS AND RESULTS: To evaluate, in LVF patients, the correlation between BNP and lung diffusion parameters at rest and during exercise, we studied 17 severe LVF patients, 13 moderate, and 10 normals measuring BNP and lung diffusion parameters before, at the end, and 1 hour after a 10-minute high-intensity constant-workload exercise. At rest, a significant correlation exists between BNP and lung diffusion parameters. Resting BNP, DLCO, and DM correlate with peak oxygen consumption (P < .0001 for all analyses). With exercise, BNP increase is significant (severe LVF 180 +/- 49 pg/mL, moderate 68 +/- 58, normals 18 +/- 12); differently, only in severe LVF, with exercise, DLCO (-1.1 +/- 0.7 mL/mm Hg/min, P < .0001) and DM (-6.4 +/- 2.8, P < .0006) decrease. One hour after exercise, only in severe LVF, BNP is still higher than at rest, while DLCO, DM, and DM/Vc are lower. Significant correlations are observed between BNP and DM changes during exercise and recovery (P < .0001) in severe LVF. CONCLUSIONS: In severe LVF, BNP changes during exercise correlate with simultaneous reductions in DM, suggesting that BNP increase and pulmonary edema formation could be related.

Emergency department evaluation and management of peripartum cardiomyopathy.

Peripartum cardiomyopathy (PPCM) affects 1000-1300 women in the United States each year. We present three cases of PPCM seen in our Emergency Department (ED) that cover the entire spectrum of disease from mild heart failure to sudden cardiac death. Without previous heart disease, these women develop cardiomyopathy with impairment of left ventricular function in the last month of pregnancy, or during the first 5 months postpartum. The etiology of PPCM is not clear, although various mechanisms have been proposed, including infection, autoimmune response, prolonged tocolysis during labor, and maladaptive responses to the hemodynamic changes of pregnancy. The initial presentation of these patients is frequently to the ED. The differential diagnosis and key characteristics of PPCM are discussed. ED management should focus on three elements: reduction in pre-load, reduction in afterload, and increase in inotropy. Key differences between the antepartum and postpartum states are highlighted.

Unique presentation of cricoid cartilage fracture causing intermittent dyspnea without preceding trauma.

Cricoid cartilage fracture is generally caused by significant neck trauma and causes continuous dyspnea, neck pain, or hoarseness developing immediately after the traumatic episode. A 69-year-old woman without any history of trauma was admitted to our hospital with intermittent dyspnea. Six months before admission she had started to complain of dyspnea occurring several times a month without warning, improving spontaneously within a few hours without treatment. Her primary care doctor diagnosed asthma and she was treated with inhaled short-acting beta agonists and glucocorticoids, without improvement. On initial evaluation at our hospital, the cause of dyspnea was unclear. Laryngoscopy was performed, which excluded vocal cord dysfunction. A further attack of dyspnea occurred on the fourth admission day. Stridor was evident during the attack, and bronchoscopy revealed subglottic narrowing of the trachea on both inspiration and expiration with no mass or foreign objects. Computed tomography (CT) of the neck revealed cricoid cartilage fracture causing airway narrowing and dyspnea. She was orally intubated, and tracheostomy was performed 2 weeks later to maintain her airway, which resolved her dyspnea. This patient's presentation was unique in two aspects. First, there was no history of trauma that may cause her cricoid cartilage fracture. Second, her symptoms of dyspnea were intermittent rather than continuous. These aspects led to suspicions of other diseases such as asthma or vocal cord dysfunction, thus delaying the diagnosis. Cricoid cartilage fracture should be considered in patients with dyspnea of unknown cause, irrespective of continuous or intermittent symptoms and preceding traumatic episodes.

Prognostic Significance of Longitudinal Clinical Congestion Pattern in Chronic Heart Failure: Insights From TIME-CHF Trial.

BACKGROUND: The relationship between longitudinal clinical congestion pattern and heart failure outcome is uncertain. This study was designed to assess the prevalence of congestion over time and to investigate its impact on outcome in chronic heart failure. METHODS: A total of 588 patients with chronic heart failure older than 60 years of age with New York Heart Association (NYHA) functional class ≥II from the TIME-CHF study were included. The endpoints for this study were survival and hospitalization-free heart failure survival. Orthopnea, NYHA ≥III, paroxysmal nocturnal dyspnea, hepatomegaly, peripheral pitting edema, jugular venous distension, and rales were repeatedly investigated and related to outcomes. These congestion-related signs and symptoms were used to design a 7-item Clinical Congestion Index. RESULTS: Sixty-one percent of patients had a Clinical Congestion Index ≥3 at baseline, which decreased to 18% at month 18. During the median [interquartile range] follow-up of 27.2 [14.3-39.8] months, 17%, 27%, and 47% of patients with baseline Clinical Congestion Index of 0, 1-2, and ≥3 at inclusion, respectively, died (P <.001). Clinical Congestion Index was identified as an independent predictor of mortality at all visits (P <.05) except month 6 and reduced hospitalization-free heart failure survival (P <.05). Successful decongestion was related to better outcome as compared to persistent congestion or partial decongestion (log-rank P <0.001). CONCLUSIONS: The extent of congestion as assessed by means of clinical signs and symptoms decreased over time with intensified treatment, but it remained present or relapsed in a substantial number of patients with heart failure and was associated with poor outcome. This highlights the importance of appropriate decongestion in chronic heart failure.

Dyspnoea versus fatigue: additional prognostic information from symptoms in chronic heart failure?

BACKGROUND: In non-heart failure populations, dyspnoea reported by the patient as the reason for stopping an exercise test is associated with a worse prognosis than fatigue. Patients with chronic heart failure (CHF) have exercise limitation due to breathlessness or fatigue, but it is unclear whether one symptom confers an adverse prognosis over the other. METHODS: Consecutive CHF patients underwent exercise testing with metabolic gas exchange. Upon stopping exercise the dominant symptom reported by the patients was recorded. Survival analyses were performed to establish predictors of mortality and relationships between symptoms and objective measures of exercise capacity. RESULTS: Data were analysed on 271 patients (219 men), mean age 67 (10) years, mean left ventricular ejection fraction 32 (8)%, and median follow-up 59 months (interquartile range 38). There were no differences in exercise variables, sex, NYHA class, body mass index and medical therapy between fatigued and dyspnoeic patients. At the censor date 92 (34%) patients had died. Deceased patients had a lower peak oxygen consumption (17.2 (4.6) versus 20.3 (5.6); p=0.0028). Although NYHA class was related to death at 36 months (chi2 value=7.3, p=0.026), reason for stopping was not (chi2 value=0.57, p=0.45). CONCLUSION: Unlike in non-heart failure populations, dyspnoea as the reason for stopping an exercise test in CHF subjects is not associated with increased mortality. CHF patients should be assessed for treatments such as cardiac resynchronisation therapy by the degree of exercise intolerance, not the nature of their symptoms.

[Gastroesophageal reflux: a possible cause of paroxysmal laryngeal dyspnea]. / La dyspnée laryngée paroxystique: une manifestation extra-oesophagienne peu connue du reflux gastro-oesophagien.

An important number of patients are referred to the ENT specialist because of extraoesophageal manifestations of gastroesophageal reflux. The most alarming symptom is a paroxysmal dyspnea secondary to a laryngospasm. The patients report a laryngeal choking sensation associated to an aphonia. We report three cases of laryngospasm secondary to acid gastric reflux. The diagnosis was made with the clinical history, a laryngeal examination and a 24-hour pH-monitoring enabled us to demonstrate a clear temporal relation between the reflux episodes and the choking episodes. In conclusion, the pharyngo-laryngeal reflux is a possible cause of laryngospasm. In our three patients, a high dose antiacid treatment was efficient to bring a lasting relief of the symptoms.