Cor Triatriatum sinester / Cor Triatriatum Sinester

ResumenEl cor triatriatum sinester es una anormalidad cardiaca congénita poco frecuente, que constituye del 0,1 al 0,4% de todas las malformaciones cardiacas. Se caracteriza por una división anómala de la aurícula izquierda por una membrana fibromuscular, la cual genera dos cavidades que se comunican por uno o más orificios con distinto grado de obstrucción.Se asocia con otras anomalías cardiacas, principalmente con comunicación interauricular. Se puede presentar en la infancia, cuando suele ser fatal, o más tardíamente en el adulto, como insuficiencia cardiaca, o incluso cursar de forma asintomática, dependiendo del tamaño de los orificios y del gradiente de presión entre las cavidades. En este artículo se presenta el caso de un masculino de 30 años, sin patologías crónicas, quien consulta por disnea de grandes esfuerzos en los últimos 6 meses, a quien se le diagnostica por medio del ecocardiograma transtorácico y transesofágico un cor triatriatum sinister.

AbstractCor Triatriatum Sinister is a rare congenital heart defect, representing 0.1 to 0.4% of all cardiac malformations. This condition is characterized for an abnormal division of the left atrium by a fibromuscular membrane that generates two cavities, which are connected by one or more orifices with different degrees of obstruction. This condition is associated with other cardiac abnormalities mainly related to atrial septal defects. Its presentation in pediatric patients is often lethal, in adults it may appear as heart failure symptoms or as an asymptomatic finding in echocardiography depending on the pressure gradient between cavities. We present a case report of a 30 years old male without chronic conditions, who complained of dyspnea with strenuous physical activity occurring in the last 6 months, who was diagnosed with a cor triatriatum sinister by the use of transthoracic and transesophageal echocardiogram.

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.

Miocardiopatía no compactada / Non-compaction cardiomyopathy

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Association between sleep apnea and overnight hemodynamic changes in hospitalized heart failure patients with and without paroxysmal nocturnal dyspnea.

BACKGROUND: Paroxysmal nocturnal dyspnea (PND) is a common symptom for patients with acute decompensated heart failure (ADHF). Some symptoms of PND are similar to those of sleep apnea (SA) which might be associated with overnight worsening hemodynamics in failing hearts. However, the association between PND, SA, and overnight change in hemodynamics in patients with heart failure remains uncertain. METHODS: We studied 28 consecutive patients with reduced ejection fraction who were hospitalized with ADHF. Plasma atrial natriuretic peptide (ANP) levels were measured before and after overnight sleep study. PND was defined as having an episode of PND prior to hospitalization for ADHF. RESULTS: Ten (36%) patients had a history of PND. Respiratory disturbance index (the frequency and severity of sleep apnea) was an independent factor associated with a history of PND (odds ratio 1.24, 95% confidence interval 1.05-1.47, p=0.011). In those without PND, plasma ANP levels decreased from before sleep to after waking, whereas in those with PND it increased (p=0.011). In addition, overnight change in plasma ANP levels was independently associated with respiratory disturbance index (p=0.035). CONCLUSION: These results thus suggest that in patients with ADHF, SA might be a predisposing cause of PND in association with overnight worsening hemodynamics.

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).

Value of orthopnea, paroxysmal nocturnal dyspnea, and medications in prospective population studies of incident heart failure.

Prospective population studies of incident heart failure (HF) are often limited by difficulties in assembling HF-free cohorts. In this study, public-use copies of the Cardiovascular Health Study (CHS) data sets were used to determine the sensitivity, specificity, and positive and negative predictive values of orthopnea and paroxysmal nocturnal dyspnea (PND), with and without the use of medications used in CHS HF criteria (diuretics plus digoxin or vasodilators), in the diagnosis of prevalent HF and in the assembly of a relatively HF-free population. Of the 5,771 community-dwelling older adults aged > or =65 years, 803 had orthopnea, 660 had PND, 1,075 had either symptom, 388 had both symptoms, 547 were using HF medications, and 4,315 had neither symptom and were not using HF medications. Definite HF was centrally adjudicated in 272 participants. The sensitivity, specificity, and positive and negative predictive values for either orthopnea or PND were 52% (95% confidence interval [CI] 46% to 58%), 83% (95% CI 82% to 84%), 13% (95% CI 11% to 15%), and 97% (95% CI 97% to 98%), respectively, and those for either orthopnea or PND or the use of HF medications were 77% (95% CI 72% to 82%), 77% (95% CI 76% to 79%), 14% (95% CI 13% to 16%), and 99% (95% CI 98% to 99%), respectively. In conclusion, only <20% of those with either orthopnea or PND had definite HF, which limits their usefulness in the diagnosis of prevalent HF in the community. However, nearly 99% (negative predictive value) of those with neither symptom nor using HF medications also did not have HF, which may be useful as a simple and inexpensive tool in assembling relatively HF-free cohorts for prospective population studies of incident HF.

Signo de Westermark como sospecha de tromboembolismo pulmonar / Westermark sign and suspicion of pulmonary thromboembolism

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Novel mutation in KCNA1 causes episodic ataxia with paroxysmal dyspnea.

Episodic ataxia type 1 (EA1) is an autosomal-dominant neurological disease caused by point mutations in the potassium channel-encoding gene KCNA1. It is characterized by attacks of ataxia and continuous myokymia. Respiratory muscle involvement has not been previously reported in EA1. We clinically evaluated a family with features of EA1 and paroxysmal shortness of breath. Coding and flanking intronic regions of KCNA1 were sequenced. We identified a novel 3-nucleotide deletion mutation in KCNA1 in the affected individuals. Our findings of a deletion mutation with unusual respiratory muscle involvement expand the genetic and clinical spectrum of EA1.

Insuficiencia respiratoria aguda como manifestación inicial de esclerosis lateral amiotrófica / Acute respiratory failure as initial manifestation of amyotrophic lateral sclerosis

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Miocardiopatía chagásica: una causa de insuficiencia cardiaca de origen infeccioso / Chagasic myocardiopathy: an infectious etiology of congestive heart failure

Se presenta el caso de un paciente latinoamericano con una miocardiopatía dilatada secundaria a una enfermedad de Chagas crónica tratada satisfactoriamente con medidas de soporte cardiológico. La intención es aportar una nueva etiología de insuficiencia cardiaca hasta ahora poco frecuente en nuestro medio pero que posiblemente vaya a aumentar debido a las corrientes migratorias actuales

We present a case report to Latin-American patient with dilated cardiomyopathy due to chronic Chagas´ disease which treat with usually cardiologic support. We report a new aetiology of heart failure uncommon in our country but it would rise due to immigration from endemic countries

Disnea paroxística durante el sueño

La disnea paroxística nocturna o durante el sueño se ha considerado durante muchos años como un síntoma específico de la insuficiencia cardiaca congestiva con fallo ventricular izquierdo y su existencia era además un signo de mal pronóstico. Con los estudios respiratorios durante el sueño se ha podido comprobar que las apneas, sean centrales u obstructivas, juegan un papel fundamental en este trastorno. Asimismo se ha podido demostrar que pacientes con otras enfermedades, como el asma bronquial, la EPOC, las restrictivas pulmonares o las neuro-musculares, tienen las primeras alteraciones respiratorias durante el sueño y por tanto pueden despertarse durante la noche con sensación de falta de aire. Para facilitar su compresión propongo la denominación de "choking" nocturno al despertar brusco con disnea. En el interrogatorio clínico hay que sospechar, en primer lugar, la obstrucción de la vía respiratoria alta que aparece en la apnea obstructiva del sueño, la hipertrofia amigdalar o macroglosia. Otras etiologías son más difíciles de detectar, como la existencia de un reflujo gastro-esofágico durante la noche, crisis de terror, pánico y otras parasomnias (AU)

Relationship between bronchial hyperreactivity and symptoms of cardiac asthma in patients with non-valvular left ventricular failure.

To determine whether a relationship exists between bronchial hyperreactivity and cardiac asthma, which is commonly observed in patients with left heart failure, a methacholine inhalation test was performed in 15 patients with stable left ventricular failure (LVF) and 10 normal subjects. The subjects were divided into 3 groups based on symptoms of nocturnal coughing and/or wheezing in acute exacerbation of LVF. Group A consisted of 8 patients with nocturnal coughing and/or wheezing. Group B consisted of 7 patients without such symptoms, and Group C consisted of the 10 age-matched normal controls. Eleven of the 15 patients with LVF showed a significant increase in respiratory resistance in the methacholine inhalation test, as opposed to none of the normal subjects. The median cumulative dose which produced a 35% decrease in respiratory conductance (PD35Grs) was significantly lower in Group A than in Group B (1.45 log units and 1.90 log units, respectively, p < 0.05). The results of pulmonary function tests were not significantly different between Groups A and B. The minimum cumulative dose required to initiate a decrease in respiratory conductance from the baseline, as an index of bronchial sensitivity to methacholine, was significantly correlated with DLCO/VA (r = 0.710, p < 0.01). We conclude that bronchial hyperreactivity is responsible for cardiac asthma and that it might be related to pulmonary interstitial changes in stable patients with non-valvular LVF.