"Its all about the fall", JACC case report revisits the classical dilemma of syncope vs. seizure.
Figure: 1) Before atrioventricular block (red arrow, “start of block”), there is slight PR interval prolongation (red bracket: PR interval, 170 ms; blue bracket: PR interval, 240 ms). There is no PP interval prolongation before block (solid red line), and there is PP interval shortening during block (dotted red line). The duration of atrioventricular block measures 22.8 s (vertical arrows, “start of block “to “end of block”). (2) Period of complete heart block without any ventricular rhythm. (3) Complete heart block with 2 ventricular escape beats, which are slightly wider than the patient’s conducted QRS complexes (blue arrows). (4) Junctional escape beats, which match the patient’s conducted QRS complexes (green arrows), followed by sinus rhythm with 2:1 conduction (P waves, not labeled because of motion artifact; conducted QRS complexes, orange arrows). (5) Sinus rhythm with 1:1 conduction (P waves, yellow arrows; conducted QRS complexes, orange arrows). Note that sinus rate has increased from 68 to 115 beats/min by the end (blue box), denoting a physiological sympathetic response to pathological atrioventricular block.
Differential diagnosis and ascertainment of the cause of transient loss of consciousness (TLOC) is both art and science for practicing physicians. The differentiation between syncope and seizures is often marred by paucity of detailed history and definite clinical examination findings.
A recent JACC case report describes one such case of convulsive TLOC where the initial diagnosis of psychogenic seizures was incorrect, but a fortuitously captured event on telemetry yielded the diagnosis: extrinsic idiopathic atrioventricular block.
A 62-year-old woman presented with multiple convulsive episodes corresponding to TLOC. Witnesses de4scribed an event lasting approximately 30 s during which the patient was sitting at a table, developed arm shaking, and then fell to the floor. She then had 2 more episodes of TLOC with jerking of her limbs, the second of which occurred during ambulation.
She denied any prodrome, including palpitations, chest pain, lightheadedness, nausea, or flushing, and any preceding aura, incontinence, tongue biting, or up-rolling of her eyes during events, although she did have confusion on awakening. Physical examination showed benign findings, including normal heart sounds and the absence of carotid bruit or recurrent symptoms with carotid massage or arm exercises.
The relevant laboratory tests, ECG, electroencephalogram, echocardiogram and brain magnetic resonance imaging were all unremarkable. Neurological consultation suggested psychogenic nonepileptic seizures (PNES) as the possible diagnosis.
But during the same admission, during telemetry, the patient experienced a witnessed recurrent event. An abrupt atrioventricular (AV) block (AVB) developed, followed by TLOC, and then convulsions. Telemetry reflected the physiology, including motion artifact corresponding to convulsions that occurred after the onset of AVB. AV conduction spontaneously resumed, and the patient awoke in a confused state. (Figure)
Transcutaneous pacing pads were placed, and an emergent transvenous pacing wire was inserted. On the basis of telemetry findings, through exclusion of other causes, a diagnosis of extrinsic idiopathic AVB (EI-AVB) was made. The patient later underwent dual-chamber pacemaker implantation programmed in the Managed Ventricular Pacing (MVP) mode.
At 3 months of follow-up, the patient had no syncope. However, she had 7 ventricular pacing events, with intrinsically conducted ventricular rates of 77 to 98 beats/min and AV delays of 130 to 160 ms immediately before ventricular pacing, consistent with episodes of EI-AVB treated with ventricular pacing. The MVP feature in this patient elucidates the necessity of pacemaker therapy in a patient with a very low ventricular pacing burden.
Despite markedly different pathophysiological characteristics, the clinical presentations of syncope and seizure can be difficult to differentiate from each other, particularly when patient-provided history is limited by loss of consciousness and episodes may be unwitnessed. In fact, misdiagnosis in TLOC may be as prevalent as 30% and is associated with increased morbidity.
Paroxysmal AVB can be divided into 3 subtypes:
1. Extrinsic vagal AVB (EV-AVB), also known as vasovagal syncope or neurocardiogenic syncope),
2. Intrinsic AVB (I-AVB, also known as Stokes-Adams syndrome) and,
3. EI(Extrinsic intrinsic) -AVB.
The patient's telemetry tracings excluded the more common EV-AVB and I-AVB. Although there was subtle PR interval prolongation on the last conducted beat, there was no PP interval prolongation before the block, as would be found in EV-AVB.
Contrary to what is often observed in I-AVB, there was no evidence of the following: native conduction disease (e.g., bundle branch block); an initiating premature atrial or ventricular complex, causing conduction tissue to become unexcitable secondary to slow partial depolarization and sodium channel inactivation (i.e., phase 4 block); or a subsequent ventricular complex resetting the membrane potential, thus prompting resolution of AVB.
Finally, the pattern observed was not consistent with phase 3 block, where a premature beat or faster atrial rate leads to AVB. Thus, the diagnosis of EI-AVB was made.
EI-AVB is an underrecognized cause of TLOC that occurs in patients with low baseline circulating plasma adenosine levels (APLs). The low APL renders patients hypersensitive to surges in endogenous adenosine, which induces AVB. A higher level of suspicion of uncommon causes, long-term ambulatory monitoring, and improved predictive tools to help guide work-up for TLOC may help reduce misdiagnosis and underdiagnosis.
Source: JACC Case Reports: Epublished DOI: 10.1016/j.jaccas.2021.04.011
