Sudden Death and Staged Therapy for Hemodynamic Stabilization in Patients Enrolled in a Heart Transplantation Program

To investigate the impact of staged therapy for advanced heart failure on therapeutic endpoints, 236 consecutive patients (coronary artery disease/dilated cardiomyopathy in 61/175 patients, left ventricular ejection fraction 14%± 5%, New York Heart Association Class IIl/IIIIV in 102/79/55 patients, respectively) with advanced heart failure were prospectively followed. One hundred thirtyseven patients enrolled from January 1989 to December 1991 were treated conventionally with digoxin, furosemide, and low dose angiotension converting enzyme (ACE) inhibition. Patients refractory to this therapy underwent urgent heart transplantation. Ninetynine patients enrolled from January 1992 to August 1993 underwent staged therapy: stage 1: maximal tolerated ACE inhibition; stage 2: therapy with PGE₁ for preand afterload reduction to achieve hemodynamic stabilization; or stage 3: refractory patients bridged to heart transplantation with continuous outpatient dobutamine. Sudden death was defined as death within 1 hour of symptoms if heart failure symptoms remained stable over the previous 7 days. Conventionally treated patients were followed for 10 ± 9 months; patients who underwent staged therapy for 9 ±5 months. In the group of patients that underwent standard therapy, 39 of 137 (28%) patients died: 5 (13%) deaths occurred suddenly, and death due to progressive pump failure occurred in the remaining 34 (87%) patients. In the group of patients that underwent staged therapy, 25 of 99 (25%) patients died: 13 (52%) deaths occurred suddenly, and 12 (48%) deaths occurred due to progressive pump failure. Thus, patients who underwent staged therapy were at increased risk for sudden death (P = 0.01, relative risk 3.4, 95% confidence interval 1.2–9.7) but were at lower risk for death from pump failure (P = 0.009, relative risk 0.44, 95% confidence interval 0.22–0.84). In patients who underwent therapy with continuous outpatient PGE₁ (n = 7) or dobutamine (n= 21), risk for sudden death (P = NS by log rank test) did not increase. In conclusion, staged therapy significantly reduced death from pump failure; however, patients who could be stabilized and considered too well for heart transplantation were at increased risk for sudden death. Thus, overall survival did not improve. Of note, outpatient dobutamine did not increase the risk for sudden death.     

Pityriasis rubra pilaris in the setting of HIV infection: clinical behaviour and association with explosive cystic acne

The development of pityriasis rubra pilaris (PRP) in three patients with human immunodeficiency virus (HIV) infection is described. Two of the patients had onset of severe generalized cystic acne concomitant with their development of PRP. PRP and acne conglobata should be added to the group of cutaneous disorders that can present in a more virulent manner in the setting of HIV infection. The association of cystic acne with PRP and their response to treatment are discussed.     

The human type II collagen gene (COL2A1) assigned to 12q14.3

A cosmid clone containing the entire human type II α1 collagen gene ( COL2A1 ) was used as probe in the Southern analysis of DNA from a panel of human/hamster somatic cell hybrids containing different portions of human chromosome 12. Two of the hybrids exhibited a similar terminal deletion q14.3→qter, but one was positive for the gene while the other was negative. Therefore, the gene must reside in the region q14.3.     

Response to Atrial Extrastimulus During Supraventricular Tachycardia: What Is the Mechanism?

Arrhythmia Rounds . We describe a case illustrating the potential challenges in distinguishing AV nodal reentry tachycardia (AVNRT) from automatic junctional tachycardia (JT). While an early atrial extrastimulus advanced the next His and ventricular depolarization without tachycardia termination, suggesting JT, other features indicated the correct diagnosis of AVNRT. This teaching case demonstrates a novel exception to a recently reported diagnostic pacing maneuver and illustrates the importance of considering response to multiple maneuvers in reaching a diagnosis of SVT mechanism. (J Cardiovasc Electrophysiol, Vol. 24, pp. 359‐363, March 2013)