RESUMO
A 49-year-old woman presented with exercise-induced chest discomfort during long-distance running that was occasionally present during rest. Significant coronary artery disease was excluded and a diagnosis of "painful left bundle branch block (LBBB) syndrome" was made after correlation of LBBB aberrancy with symptoms during Holter monitoring. The patient underwent confirmatory testing consisting of rapid atrial pacing below and above 130 beats per minute, the rate cut-off for LBBB manifestation. His bundle pacing implantation was performed resulting in both non-selective and selective morphologies depending on output, both of which manifested with a painless narrow QRS regardless of rate. She was rendered completely pain free during long-distance running and remains so 6-months later. Her pain at rest, now thought to be due to severe anxiety secondary to her painful LBBB, has also subsided. Exercise-induced, painful LBBB is a rare phenomenon that manifests as chest discomfort when LBBB is present. This disease is frequently misdiagnosed as coronary angina, has limited medical treatment options, and can be disabling. HBP is an attractive treatment for this syndrome in an effort to avoid electromechanical dyssynchrony, the presumed mechanism of discomfort. This case report adds to the growing literature of painful LBBB syndrome and its effective treatment with HBP, with the added caveat that it can present with persistent symptoms at rest, in the setting of enhanced anxiety. HBP should be considered early on in the treatment of such patients.
RESUMO
OBJECTIVE: To characterize patterns of care at the end of life for children and young adults with life-threatening complex chronic conditions (LT-CCCs) and to compare them by LT-CCC type. STUDY DESIGN: Cross-sectional survey of bereaved parents (n = 114; response rate of 54%) of children with noncancer, noncardiac LT-CCCs who received care at a quaternary care children's hospital and medical record abstraction. RESULTS: The majority of children with LT-CCCs died in the hospital (62.7%) with more than one-half (53.3%) dying in the intensive care unit. Those with static encephalopathy (AOR, 0.19; 95% CI, 0.04-0.98), congenital and chromosomal disorders (AOR, 0.28; 95% CI, 0.09-0.91), and pulmonary disorders (AOR, 0.08; 95% CI, 0.01-0.77) were significantly less likely to die at home compared with those with progressive central nervous system (CNS) disorders. Almost 50% of patients died after withdrawal or withholding of life-sustaining therapies, 17.5% died during active resuscitation, and 36% died while receiving comfort care only. The mode of death varied widely across LT-CCCs, with no patients with pulmonary disorders dying receiving comfort care only compared with 66.7% of those with CNS progressive disorders. A majority of patients had palliative care involvement (79.3%); however, in multivariable analyses, there was distinct variation in receipt of palliative care across LT-CCCs, with patients having CNS static encephalopathy (AOR, 0.07; 95% CI, 0.01-0.68) and pulmonary disorders (AOR, 0.07; 95% CI, 0.01-.09) significantly less likely to have palliative care involvement than those with CNS progressive disorders. CONCLUSIONS: Significant differences in patterns of care at the end of life exist depending on LT-CCC type. Attention to these patterns is important to ensure equal access to palliative care and targeted improvements in end-of-life care for these populations.