Predictors of COVID-19 outcomes in adult congenital heart disease patients - anatomy versus function.

BACKGROUND: It is unclear whether patients with adult congenital heart disease (ACHD) should be considered as an increased risk population with poor outcomes when suffering from COVID-19. AIMS: This study aimed to collect clinical outcome data and to identify risk factors of a complicated course of COVID-19 among ACHD patients. METHODS: Among all outpatients who came to medical attention via telemedicine or direct physician contact at our institution between September 1, 2020 and March 31, 2021, we included all with a COVID-19 diagnosis. The incidence of COVID-19, a clinical course of the disease, and outcome were determined. RESULTS: One hundred and four (8.7%) out of 1 197 patients who were seen at our outpatient clinic for ACHD patients met the definition of COVID-19. Most of them reported a mild course of COVID-19 (99 [95.5%]). Five patients (4.5%) experienced severe symptoms and needed hospitalization. Two patients (1.9% of all with a confirmed diagnosis, 40% with severe infection) died. In the multivariable analysis, decreased systemic ventricular systolic function and any significant valve stenosis were predictors of a complicated disease course. CONCLUSIONS: Our study confirmed previous results showing that a physiology-based model, rather than an anatomy-based model, better predicted COVID-19 outcomes among ACHD patients, which is of importance for patients and healthcare providers during the COVID-19 pandemic.

Prolonged left ventricular contraction duration in apical segments as a marker of arrhythmic risk in patients with long QT syndrome.

AIMS: Long QT syndrome (LQTS) is an inherited cardiac ion channelopathy predisposing to life-threatening ventricular arrhythmias and sudden cardiac death. The aim of this study was to investigate left ventricular mechanical abnormalities in LQTS patients and establish a potential role of strain as a marker of arrhythmic risk. METHODS AND RESULTS: We included 47 patients with genetically confirmed LQTS (22 LQT1, 20 LQT2, 3 LQT3, and 2 SCN3B) and 25 healthy controls. A history of cardiac events was present in 30 LQTS subjects. Tissue Doppler and speckle tracking echocardiography were performed and contraction duration was measured by radial and longitudinal strain. The radial strain characteristic was subdivided into two planes - the basal and the apical. Left ventricular ejection fraction and global longitudinal strain were normal in LQTS patients. Mean contraction duration was longer in LQTS patients compared with controls in regard to basal radial strain (491 ± 57 vs. 437 ± 55 ms, P < 0.001), apical radial strain (450 ± 53 vs. 407 ± 53 ms, P = 0.002), and longitudinal strain (445 ± 34 vs. 423 ± 43 ms, P = 0.02). Moreover, contraction duration obtained from apical radial strain analysis was longer in symptomatic compared with asymptomatic LQTS mutation carriers (462 ± 49 vs. 429 ± 55 ms, P = 0.024), as well as in subject with mutations other than LQT1 considered to be at higher risk (468 ± 50 vs. 429 ± 49 ms, P = 0.01). CONCLUSION: Myocardial contraction duration is prolonged for both radial and longitudinal directions in LQTS patients. Regional left ventricular function analysis may contribute to risk stratification. Apical radial deformation seems to select subjects at higher risk of arrhythmic events.

Anterior mitral leaflet length and mitral annulus diameter impact the echocardiographic outcome after isolated myectomy.

BACKGROUND: Myectomy remains the standard surgical treatment of patients with hypertrophic cardiomyopathy (HOCM). New surgical methods developed in the last decades mainly address the mitral valve and are controversial because of their conflicting assumptions. This study assesses the influence of anterior mitral valve leaflet (AML) length and the anterior-posterior diameter of the mitral annulus (MAD) on dynamic left ventricle outflow tract obstruction and mitral regurgitation (MR) after extended myectomy. METHODS: We retrospectively analysed the transthoracic echocardiograms (TTE) of 36 patients. AML length and MAD were obtained from TTE performed before the operation. The greatest maximal left ventricle outflow tract (LVOT) gradient and MR registered in follow-up were analysed. After surgery, patients were divided into two groups; those with moderate or milder MR and/or an LVOT gradient < 30 mmHg (responders), and those with more than moderate MR and/or an LVOT gradient ≥30 mmHg (non-responders). RESULTS: Patients in responders group had significantly longer AML: 32.3 ± 2.3 mm vs 30.0 ± 3.8 mm (p = 0.03) [parasternal long axis view - PLAX view], 25.9 ± 2.3 mm vs 23.5 ± 2.7 mm (p = 0.008) [four chamber view - 4CH view] and larger anterior-posterior mitral annulus diameter 28.1 ± 2.8 mm vs 25.4 ± 3.2 mm (p = 0.011) than those in non-responders group. Among all analysed patients longer anterior mitral leaflet was correlated with lower postoperative LVOT gradient when measured in PLAX view (p = 0.02) and lower degree of MR due to systolic anterior motion (SAM) when measured in 4CH view (p = 0.009). Greater [AML x mitral annulus] ratio correlated with lower postoperative LVOT gradient in both projections: 4CH (p = 0.025), PLAX (p = 0.012). There was significant reduction in NYHA Class after surgery (p = 0.000). There were no significant differences in NYHA class after surgery (p = 0.633) neither in NYHA class reduction (p = 0.475) between patients divided into responders and non-responders group according to echocardiographic parameters. CONCLUSIONS: Patients with a longer AML and a greater diameter of the mitral annulus are less likely to have mitral regurgitation due to residual SAM and increased LVOT gradient after an extended myectomy. Division of patients according to echocardiographic criteria into responders and non-responders was not in concordance with clinical improvement. TRIAL REGISTRATION: Retrospective study. Approved by ethics committee (IK-NPIA-0021-21/1763/19) at 16.01.2019.

Cardiac arrest and ventricular arrhythmia in adults with Ebstein anomaly and left ventricular non-compaction.

BACKGROUND: Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC). AIM: We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA. METHODS: We conducted a retrospective search of our institutional database from 2010 to 2014 for patients with EA and reviewed patients' medical records (age, sex, clinical presentation, electrocardiographic, echocardiographic, and CMR - cardiac magnetic resonance features). We reviewed echocardiograms and CMR scans for concomitant morphological abnormalities (LVNC, PDA - patent ductus arteriosus, VSD - ventricular septal defect, ASD - atrial septal defect, mitral valve prolapse, BAV - bicuspid aortic valve, CoA - coarctation of aorta). RESULTS: The studied group consisted of 84 consecutive patients (mean age 38±15 years, 50 women) with EA. We found four patients (4.8%) with LVNC, two of them had cardiac arrest, one had VT, and one was symptomless, but had QTc prolongation in Holter recordings. Concomitant abnormalities were VSD (4.8%), PDA (1.2%), CoA (1.2%), mitral valve prolapse (1.2%), and BAV (2.4%). The most common anomaly was ASD type II - 23 patients (27.3%) and WPW - Wolff-Parkinson-White's syndrome - 9 patients (10.7%). CONCLUSIONS: Non-compaction is a notable abnormality in adult patients with EA and it may affect their prognosis. Although other concomitant lesions were more common, only patients with LVNC suffered from cardiac arrest or ventricular arrhythmia.

Collagen turnover biomarkers and systemic right ventricle remodeling in adults with previous atrial switch procedure for transposition of the great arteries.

BACKGROUND: Myocardial fibrosis is a potential pathophysiological mechanism leading to systemic right ventricular (SRV) deterioration. We hypothesize that circulating levels of collagen deposition markers are elevated in patients with SRV remodeling and this elevation may have a predictive value. METHODS: We prospectively evaluated 56 patients with D-TGA after the atrial switch procedure (mean age 25.6 ± 4.8, range 18-37 years; 67% males). Serum levels of procollagen type III amino-terminal propeptide (PIIINP), collagen type I carboxy-terminal telopeptide (CITP), procollagen type I N-terminal propeptide (PINP), matrix metalloproteinase (MMP 1, MMP 9) and a tissue inhibitor of matrix metalloproteinase (TIMP 1) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) were measured and compared with healthy controls. The relationship between these serum markers, echocardiographic and cardiac magnetic resonance parameters and the outcome at a follow-up of 61 months (range, 24-85 months) was determined. RESULTS: Compared with the healthy control group, the study group had significantly higher levels of TIMP1, PIIINP, CITP, PINP and NT-pro-BNP (p<0.05, each). The levels of PIIINP and CITP were significantly higher among patients with an SRV mass index above the mean value. The level of PIIINP was significantly higher among patients with an SRV EDV index above the mean value. CITP was significantly elevated in SRV late gadolinium enhanced (LGE) positive patients, compared to patients without SRV LGE. MMP9 and TIMP1 predicted an adverse clinical outcome on univariate Cox proportional hazard survival analysis in addition to well proven predictors of outcome (SRV EF and NYHA). CONCLUSIONS: We demonstrated a pattern of altered collagen turnover adversely related with the indices of SRV remodeling and an adverse clinical outcome in patients with SRV.

Pregnancy in women with complete transposition of the great arteries following the atrial switch procedure. A study from three of the largest Adult Congenital Heart Disease centers in Poland.

OBJECTIVE: We sought to identify maternal/neonatal and cardiovascular complications in pregnant women with complete transposition of great arteries (D-TGA) following atrial switch. METHODS: Clinical records of all women with D-TGA after the Mustard/Senning (M/S) operation who were followed at the three largest Adult Congenital Heart Disease (ACHD) centers in Poland were reviewed. RESULTS: Fifteen of the fifty-nine women followed had a total of 24 pregnancies, including two spontaneous miscarriages. Twenty-two pregnancies (92%) resulted in a live birth, 91% were by cesarean section. During 5 (23%) of the 22 completed pregnancies obstetric complications were observed (one gestational diabetes, one hypertension in pregnancy, one placenta increta and two preterm labors). The mean pregnancy duration was 37.2 weeks (range: 26-41 weeks).We observed one neonatal death due to extreme prematurity. Six (25%) children had a birth weight of ≤2500 g. None of women had severe cardiac complications during pregnancy nor in the postpartum period. CONCLUSIONS: In our study, we demonstrated a large number of obstetric complications and low birth weight in the presence of a systemic right ventricle. However, from a cardiologist's point of view pregnancy after the M/S operation was well-tolerated and relatively safe.

Global longitudinal strain may identify preserved systolic function of the systemic right ventricle.

BACKGROUND: Assessment of systemic right ventricle (RV) function is a key point in the follow-up of patients with dextroposition of the great arteries (D-TGA) after the atrial switch procedure. Cardiac magnetic resonance (CMR) is considered the reference standard but is not feasible for a considerable number of patients. Experience with the application of 2-dimensional speckle tracking echocardiography for the assessment of systemic RV function in such patients is very limited, and the cutoff strain value differentiating normal and subnormal RV function is missing. METHODS: We prospectively examined, with echocardiography and CMR, 40 patients with D-TGA (mean age, 25.6 ± 5 years; 25 men; 33 after the Senning, 7 after the Mustard procedure) who attended a tertiary adult congenital heart disease centre. RESULTS: There was a linear correlation between global longitudinal strain (GLS) and CMR systemic RV ejection fraction (EF) values (r = 0.4; P = 0.01), systemic RV fractional area change and CMR systemic RV EF (r = 0.41; P = 0.008). There was no correlation between systemic RV s', tricuspid annular plane systolic excursion or the rate of change of the systemic RV pressure with time and CMR systemic RV EF. Only 1 of 19 patients with GLS < -14.2% had CMR systemic RV EF < 45%. The GLS cutoff value of -14.2% predicted the CMR systemic RV EF of ≥ 45% with 90% specificity and 83% sensitivity. Its positive predictive value was 96%. CONCLUSIONS: The systemic RV GLS is able to discriminate between D-TGA patients after the atrial switch with, and those without, a CMR systemic RV EF of at least 45%.

"Migraine side effect" and stroke recurrence in late follow-up after transcatheter patent foramen ovale closure as secondary prevention of cryptogenic thromboembolism.

INTRODUCTION: Patent foramen ovale (PFO) is associated with cryptogenic strokes, recurrent transient neurologic deficits, sleep apnea, decompression illness and migraines with aura. AIM: We verify cryptogenic embolism recurrence after transcatheter PFO closure in patients younger than 55 years old, and determine the prevalence of migraine with aura before and after PFO closure. MATERIAL AND METHODS: We sent a questionnaire concerning the recurrence of stroke or transient ischemic attack (TIA) and the presence of migraine symptoms before and after PFO closure to 224 consecutive patients (mean age 40.9 ±9 years; 103 men; 108 patients < 40 years old, 116 patients 40-55 years old) after successful PFO transcatheter closure as secondary prevention of cryptogenic embolism. RESULTS: The mean follow-up period was 37.8 ±32.5 (median 27) months. Stroke or TIA recurred in 6 patients (2.6%), all of whom were over 40 years old at the time of closure. The median time of recurrence was 24 months. Two patients (0.89%) died, but the deaths were not related to the device nor to thromboembolism. Migraine occurred in the study group before closure in 68 (30.4%) patients. After the procedure 55 (80.9%) reported improvement or disappearance of migraine symptoms. CONCLUSIONS: Recurrent strokes after PFO closure are rare, and they occur more often in patients over 40 years old at the time of closure. The PFO closure results in migraine subsiding or symptoms noticeably ameliorating.

Coronary artery fistula in a 70 year-old woman with myocardial infarction.

Coronary artery fistulas are infrequent congenital malformations. We present the case of a patient with acute coronary syndrome and fistula between the proximal left anterior descending artery (LAD) and the pulmonary artery. The fistula was diagnosed during coronary angiography. Its presence and morphology was confirmed by a 64-slice computer tomography scan. The patient underwent a successful coronary artery bypass grafting procedure with surgical closure of the fistula.

Is the diagnostic function of pacemakers a reliable source of information about ventricular arrhythmias?

BACKGROUND: The aim of this study was to evaluate the reliability of pacemaker diagnostic function in diagnosing ventricular arrhythmias. METHODS: We compared the occurrence of ventricular ectopic beats in 51 simultaneous 24-hour electrocardiogram (ECG) recordings and pacemaker event counters printouts. The diagnostic function of a pacemaker allowed also for a qualitative assessment in 38 patients. In these cases, the occurrence of complex forms of ventricular arrhythmias was cross-checked for accelerated ventricular rhythms together with ventricular tachycardia, and triplets and couplets. The detection of at least one type of complex ventricular form of arrhythmia, diagnosed by both methods, was considered as an agreement between the methods. RESULTS: The results of ventricular ectopic beat counts differed significantly between the methods. In three (6%) patients, the results were consistent; in 20 (39%) the pacemaker underestimated results; in 28 (55%) they were overestimated. When more liberal criteria of agreement were applied, clinically significant differences were observed in 24 (47%) patients; in seven (29%) patients the count made by the pacemaker was lowered; and in 17 (71%) it was overestimated. Ventricular tachycardias were recorded in 24-hour ECG in eight patients. In three, they were identified by the pacemaker diagnostic function. In five, the pacemaker did not recognize tachycardia (because of its frequency being below 120/min). In nine, tachycardia was recognized falsely. The sensitivity in ventricular tachycardia diagnosis by pacemaker diagnostic function was 38%, specificity - 70%, the value of a positive result - 25%, negative - 81%. CONCLUSIONS: The evaluation of ventricular arrhythmias by pacemaker cannot serve as the only reliable diagnostic method of arrhythmias. The presence of a large number of sequences that may correspond to ventricular arrhythmia or failure to sense, should result in verification via 24-hour ECG monitoring.