Introduction
Patients and Methods
Patients
Non-invasive Work-Up
Electrophysiological Study and Catheter Ablation
Endomyocardial Biopsy
Follow-Up
Statistical Analysis
Results
Patients
Variable | Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 |
---|---|---|---|---|---|---|---|---|
Patients’ demographics and symptoms | ||||||||
Age at AFL-onset | 10.1 years | 14.9 years | 13.5 years | 11.2 years | 10.8 years | 11.9 years | 15.4 years | 13.9 years |
Age at EPS | 10.4 years | 15 years | 16.7 years | 11.8 years | 12.3 years | 12.1 years | 16.4 years | 16.4 years |
Frequency of AFL | 5 times per month since 3 months | Recurrent episodes since a few months | Every 3 months since 3 years | Total of 3 episodes since 6 months | Every 3 months since 1.5 years | Recurrent episodes, sometimes daily, since a few months | Total of 2 episodes since 1 year | Total of 4 episodes since 2.5 years |
Duration of AFL | Non-sustained | Non-sustained | Sustained | Sustained | Sustained | Sustained | Sustained | Sustained |
Syncope | No | No | No | No | Yes | No | Yes | No |
Other symptoms | Palpitations | Dizziness | Palpitations | Palpitations | Palpitations | Chest pain, dyspnea and dizziness | Dizziness | Palpitations, dyspnea, pressure on the chest |
Non-invasive findings | ||||||||
ECG in sinus rhythm prior to ablation | Incomplete RBBB (QRS duration 88 ms) | Incomplete RBBB (QRS duration 96 ms) | Normal | Normal | Normal | Normal | Normal | Normal |
Holter monitors | Two episodes of non-sustained AFL | Normal | Normal | 3 single SVES, 3 single PVC | Intermittent 2nd degree AV block | AFL, single PVC | Normal | Normal |
Echocardiography | Normal | Normal | Normal | Normal | Normal | Normal | Normal | Normal |
CMR | n.e | Normal | n.e | n.e | Normal | n.e | n.e | Normal |
Invasive findings | ||||||||
Hemodynamics and angiographies | Normal | Normal | Increased trabecularization of both ventricles, slightly reduced LV function, EF 51% (normal > 52%) | Normal | Normal | Elevated LVEDP (16 mHg; normal ≤ 12 mmHg) | Normal | Elevated LVEDP (13 mmHg; normal ≤ 12 mmHg) |
Additional findings during EPS | None (AFL not inducible) | None (AFL not inducible) | Left AT, AF (degenerated from AFL) | AVNRT, AF (degenerated from AFL) | Additional FAT inducible once | FAT and AF (degenerated from AFL) | None | None |
Results of endomyocardial biopsies | n.e | Minor fibrolipomatous changes consistent with early ARVC | Initial biopsy: normal repeat biopsy after 43 months: chronic lymphocytic myocarditis | Normal | Normal | Initial biopsy: chronic myocarditis, HHV6 persistence repeat biopsy after 16 months: result unchanged | n.e | State after myocarditis |
Follow-up | ||||||||
Duration of follow-up | 18 months | 16 months | 47 months | 31 months | 117 months | 75 months | 24 months | 12 months |
Recurrence of arrhythmia | No | Yes, non-sustained wide QRS complex tachycardia; two weeks after EPS | Yes, monomorphic PVC (max 27% of all QRS complexes); 3 years after EPS | No | Yes, AFL; 5,7 years after EPS | Yes, 1st recurrence AFL; one day after EPS; 2nd recurrence MAT; early after 2nd EPS | No | No |
Repeat EPS | No, n.a | Yes, repeat induction of CTI conduction block; no sustained SVT and no VT inducible | Yes, ablation of left ventricular focus | No, n.a | Yes, two repeat procedures, closure of conduction gaps within the CTI | Yes, 1st repeat procedure: isolation of coronary sinus ostium, 2nd repeat procedure: RFA of MAT | No, n.a | No, n.a |
Further clinical course | n.a | Propranolol for one year, genetic testing for CPVT, HCM and ARVC normal | Monomorphic PVC reduced after 2nd EPS and Bisoprolol, left ventricular dilatation DD tachycardia-induced DD DCM | n.a | After repeat EPS and death of brother beta blocker, ICD-therapy, genetic negative familial Brugada syndrome (unspecific heterozygous DSP-gene sequence variant) | Recurrences poorly controlled with antiarrhythmic medication, free from tachycardia without any treatment after 3rd EPS | n.a | n.a |
Final diagnoses | “Lone AFL” | Not established | Chronic lymphocytic myocarditis | “Lone AFL” | Familial Brugada syndrome | Chronic myocarditis with HHV6 persistence | “Lone AFL” | State after myocarditis |
Status at end of follow-up | Free from tachycardia | On propranolol free from tachycardia, medication stopped on last follow-up visit | On bisoprolol reduced PVC (254/24 h) | Free from tachycardia | On sotalol free from tachycardia, ICD | Free from tachycardia | Free from tachycardia | Free from tachycardia |