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Pulmonary veins stenosis relief after an inappropriate radiofrequency catheter ablation of atrial fibrillation in a young non-competitive athlete.

Monaldi Arch Chest Dis. 2018 Mar 09;88(1):895

Authors: Sarubbi B, Rea G, Santoro G, Melillo E, Scognamiglio G, Russo MG

One of the major complications of radiofrequency catheter ablation (RFCA) for atrial fibrillation (AF) is pulmonary vein stenosis (PVS). The natural history of PVS, especially when it involves more than one vein, leads to severe and irreversible pulmonary hypertension with end-stage right heart failure that can require, in extreme cases, even heart-lung transplantation. We report the case of a young patient who underwent RFCA for a single lasting episode of AF and developed PVS years later. He was treated with ballon venoplasty followed by stent implantation in left pulmonary vein because of PVS relief. This reported case emphasizes the need of an adequate indication for RFCA for AF, considering the benefit-risk ratio especially in young patients with normal cardiac function.

PMID: 29557576 [PubMed - in process]

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High sensitivity cardiac troponin T and systemic right ventricular function in adults with congenitally corrected transposition of the great arteries.

Int J Cardiol. 2017 Aug 15;241:168-172

Authors: Kowalik E, Klisiewicz A, Rybicka J, Biernacka EK, Hoffman P

BACKGROUND: High sensitive troponin T (hsTnT), a marker of myocardial injury, appears to be a promising diagnostic tool in patients with congenital heart disease. However, little is known about its distribution among adults with systemic right ventricle (sRV). We aimed to assess the distribution of hsTnT concentrations in patients with congenitally corrected transposition of the great arteries (ccTGA) and to evaluate its relationship with sRV function and NT-proBNP.
METHODS: A cross-sectional study of adults with ccTGA was conducted. Patients underwent transthoracic echocardiography, hs-TnT and NTproBNP measurements. In the echocardiographic study, the sRV function was assessed qualitatively and quantitatively using fractional area change (FAC), TAPSE, myocardial performance index (MPI), systolic pulsed Doppler velocity (s') and global longitudinal strain (GLS.
RESULTS: Fifty patients with ccTGA (20F/30M) and a mean age of 34.8±13.6years (range 18-63years) were included, with 27 of them (54%) having detectable hsTnT. Patients with detectable hsTnT were older and more often had NYHA class>I. Detectable hsTnT was associated with lower FAC (0.35 vs. 0.41, p<0.01) and GLS (-14.4% vs. -17.8%, p<0.01)) and higher MPI (0.67 vs. 0.48, p<0.01)). Hs-TnT correlated weekly with NT-proBNP (r=0.38; p<0.001). The area under the curve for the detection of sRV dysfunction (FAC<0.35) was higher for hs-TnT (0.839; CI 0.713-0.952) than for NT-proBNP (0.709; CI 0.545-0.873).
CONCLUSION: HsTnT was detectable in over half of the ccTGA population and was related to the sRV function. Compared to NTproBNP, hsTnT level seems to be superior biomarker in discriminating patients with sRV dysfunction.

PMID: 28302319 [PubMed - indexed for MEDLINE]

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Mortality, rehospitalizations and costs in children undergoing a cardiac procedure in their first year of life in New South Wales, Australia.

Int J Cardiol. 2017 Aug 15;241:156-162

Authors: Lawley CM, Lain SJ, Figtree GA, Sholler GF, Winlaw DS, Roberts CL

BACKGROUND: Cardiac procedures are part of management for many children with congenital heart disease (CHD). Using population health data, this study explores health outcomes of children undergoing a cardiac procedure in the first year of life to better understand the impact of CHD on children, families and health services.
METHODS AND RESULTS: A population-based record-linkage cohort study was undertaken. Rate of cardiac procedures in the first year of life over the study period 2001-2012 in New South Wales, Australia, was steady at 2.5 children per 1000 live births, accounting for 2722 children. Excluding those with isolated closure of patent ductus arteriosus (n=416), 50% required readmission in the first year of life. Over 1/5th had an additional non-cardiac congenital anomaly. Average total cost per infant for initial procedure admission was $67,054 AUD ($63,124-$70,984) with a median length of stay (LOS) 13days (IQR 8-23). Average cost per readmission in the first year of life was $11,342 (95% CI 10,361-$12,323) with median LOS 2days (IQR 1-5). Mortality rate in the 30days following initial procedure was 3.1% (72/2306). Mortality rate by age 1year was 7.1%, and 13.8% for those who had neonatal surgery.
CONCLUSION: Risk of mortality in operatively-managed CHD extends beyond the immediate perioperative period. Children undergoing a cardiac procedure in their first year are often readmitted to hospital for both further planned procedures and unplanned reasons such as infection. These readmissions capture the significant impact of illness and pose substantial financial cost to the health system.

PMID: 28385359 [PubMed - indexed for MEDLINE]

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A novel 'splice site' HCN4 Gene mutation, c.1737+1 G>T, causes familial bradycardia, reduced heart rate response, impaired chronotropic competence and increased short-term heart rate variability.

Int J Cardiol. 2017 Aug 15;241:364-372

Authors: Hategan L, Csányi B, Ördög B, Kákonyi K, Tringer A, Kiss O, Orosz A, Sághy L, Nagy I, Hegedűs Z, Rudas L, Széll M, Varró A, Forster T, Sepp R

BACKGROUND: The most important molecular determinant of heart rate regulation in sino-atrial pacemaker cells includes hyperpolarization-activated, cyclic nucleotide-gated ion channels, the major isoform of which is encoded by the HCN4 gene. Mutations affecting the HCN4 gene are associated primarily with sick sinus syndrome.
METHODS AND RESULTS: A novel c.1737+1 G>T 'splice-site' HCN4 mutation was identified in a large family with familial bradycardia which co-segregated with the disease providing a two-point LOD score of 4.87. Twelve out of the 22 investigated family members [4 males, 8 females average age 36 (SD 6) years] were considered as clinically affected (heart rate<60/min on resting ECG). Minimum [36 (SD 7) vs. 47 (SD 5) bpm, p=0.0087) and average heart rates [62 (SD 8) vs. 73 (SD 8) bpm, p=0.0168) were significantly lower in carriers on 24-hour Holter recordings. Under maximum exercise test carriers achieved significantly lower heart rates than non-carrier family members, and percent heart rate reserve and percent corrected heart rate reserve were significantly lower in carriers. Applying rigorous criteria for chronotropic incompetence a higher number of carriers exhibited chronotropic incompetence. Parameters, characterizing short-term variability of heart rate (i.e. rMSSD and pNN50%) were increased in carrier family members, even after normalization for heart rate, in the 24-hour ECG recordings with the same relative increase in 5-minute recordings.
CONCLUSIONS: The identified novel 'splice site' HCN4 gene mutation, c.1737+1 G>T, causes familial bradycardia and leads to reduced heart rate response, impaired chronotropic competence and increased short-term heart rate variability in the mutation carriers.

PMID: 28465117 [PubMed - indexed for MEDLINE]

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PFO Closure for Cryptogenic Stroke: A Review and Clinical Treatment Algorithm.

Cardiol Rev. 2017 Jul/Aug;25(4):147-157

Authors: Singh HS, Katchi F, Naidu SS

With a high prevalence in the general population of approximately 25%, and a prevalence in the cryptogenic stroke population approaching 40%, the propensity of a patent foramen ovale (PFO) to precipitate or enable stroke, especially in young, otherwise healthy individuals, has been the subject of much debate. With proof of concept achieved via imaging modalities documenting thrombus-in-transit, and the development of minimally-invasive percutaneous approaches to closure, multiple observational studies and, more recently, several completed randomized controlled trials have sought to answer the question of when and in whom PFO closure should occur. We describe the historical context of PFO closure and review the observational and randomized control trial evidence in this field, culminating in the recent Food and Drug Administration approval of the first dedicated closure device for PFO. Guidelines and consensus statements are discussed, and a novel treatment algorithm is proposed. Future directions in PFO closure will include new devices, further data from completed and upcoming clinical trials, and potential expansion into other disease states associated with PFO.

PMID: 28574934 [PubMed - indexed for MEDLINE]

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Commentary on "Prevalence of HCV Infection in Adults with Congenital Heart Disease and Treatment with Direct Antiviral Agents".

South Med J. 2018 03;111(3):142-143

Authors: Balaguru D

PMID: 29505647 [PubMed - indexed for MEDLINE]

Intrahepatic cholangiocarcinoma after Fontan procedure in an adult with visceral heterotaxy.

Pathol Res Pract. 2018 Mar 15;:

Authors: Wang D, Marshall D, Veldtman G, Gupta A, Trout AT, Villafane J, Bove K

Hepatic dysfunction, including development of hepatocellular carcinoma and other liver lesions has been increasingly reported following Fontan procedure for congenital heart disease. We report a unique case of intrahepatic cholangiocarcinoma 28 years after a Fontan procedure in a 31year old female with heterotaxy syndrome. The subcapsular mass-forming tumor was composed of poorly differentiated tumor cells arranged in small vague glandular or slit-lumen nests, and focally fused or anastomosing large trabecular patterns within the prominent fibrotic stroma. The tumor cells with immunoreactivity to CK7, CK19, Cam5.2, COX2, EMA, BCL-2, MOC-31 and AE1/AE3, supported a diagnosis of intrahepatic cholangiocarcinoma. Focal atypical ductular proliferation within the background liver may represent a precursor lesion to this tumor. Dysmorphic cilia observed by electron microscopy examination in the background liver may suggest cholangiociliopathy in heterotaxy. MYST3 mutation at Q1388H detected in intrahepatic cholangiocarcinoma is reported for the first time.

PMID: 29559246 [PubMed - as supplied by publisher]

Mechanical circulatory support challenges in pediatric and (adult) congenital heart disease.

Curr Opin Organ Transplant. 2018 Mar 20;:

Authors: Schweiger M, Lorts A, Conway J

PURPOSE OF REVIEW: Increased miniaturization of ventricular assist devices (VADs) and new mechanical support strategies (MCS) has increased the use of MCS in the pediatric and congenital heart disease (CHD) population. This comes with the need for care providers specialized in this field to determine optimal patient and device selection, and to improve outcomes and decrease complication rates for new innovative strategies. A review of the published literature in this field is timely and relevant.
RECENT FINDINGS: There has been a rapid evolution of using adult designed continuous flow VADS to support children and adults with CHD (ACHD). Patient selection for patients with CHD is complex because of patient size and anatomical diversity and, therefore, makes decision-making complex and unique when compared to general adult practice. Outcomes for children depend on size and diagnosis with neonates with single ventricle physiology being the highest risk candidates. This also holds true for ACHD, in which VAD outcomes in patients with two ventricle physiology are comparable to non-ACHD patients.
SUMMARY: In children, there is an increased use of continuous flow devices and a growing experience with outpatient management. Patients with CHD especially when associated with single ventricle physiologies, remain a challenge when it comes to MCS/VAD placement but successful durable VAD implantation with discharge home has been reported.

PMID: 29561310 [PubMed - as supplied by publisher]

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Eccentric Left Ventricular Hypertrophy in Aortic Stenosis Caused by Unicuspid Aortic Valve.

Circ J. 2017 05 25;81(6):895-897

Authors: Higashi H, Ogimoto A, Inoue K, Tokunaga N, Iio C, Aono J, Yoshida S, Izutani H, Tabara Y, Okura T, Higaki J, Ikeda S

PMID: 28123168 [PubMed - indexed for MEDLINE]

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Pre-pregnancy counseling for women with heart disease: A prospective study.

Int J Cardiol. 2017 Aug 01;240:374-378

Authors: Cauldwell M, Steer PJ, Swan L, Patel RR, Gatzoulis MA, Uebing A, Johnson MR

BACKGROUND: Women with cardiac disease and their infants are at a greater risk of mortality and morbidity during pregnancy. Expert groups recommend preconception counseling (PCC) for all women with cardiac disease so they are made aware of these risks. We have run a specialist maternal cardiac clinic since 1996. The aim of this study was to evaluate the experience of women who have received PCC within an established multidisciplinary tertiary clinic and to establish their views regarding the counseling they received.
METHODS: Single centre prospective study using a patient questionnaire was given to women attending a specialist cardiac preconception counseling clinic from November 2015 to August 2016, with analysis of descriptive data and free text comments from the questionnaire responders.
RESULTS: 40/65 returned patient questionnaires. Prior to the consultation fewer than half felt well informed regarding how their heart disease could impact upon pregnancy but a similar proportion felt nonetheless that they would be able to have a healthy pregnancy. Women reported two main areas of concerns, their own health (whether they would survive a pregnancy) and the health of their child. 15% of women reported that these concerns had prevented them from pursuing a pregnancy. Women reported high satisfaction rates with the clinic.
CONCLUSIONS: There is an increasing demand for PCC services for women with cardiac disease; our study is the first attempt to determine both the acceptability and the impact of PCC from the patient perspective. Patients reported a high level of satisfaction with the service provided.

PMID: 28377190 [PubMed - indexed for MEDLINE]