Journal Watch

Related Articles

Voltage mapping to guide pacemaker placement in patients with CHD.

Cardiol Young. 2019 Oct;29(10):1313-1316

Authors: Dasgupta S, Fischbach P, Whitehill R

Abstract
Transvenous pacemaker implantation for sinus node dysfunction in patients with Fontan palliation presents the difficulty of finding suitable pacing tissue and the potential of causing vascular obstruction in a low-flow circuit. We describe a patient who underwent electro-anatomic voltage mapping to guide a transvenous single chamber lead within her Fontan baffle. This highlights the use of advanced mapping technologies for pacemaker implantation in complex cyanotic heart disease.

PMID: 31475646 [PubMed - indexed for MEDLINE]

Related Articles

Accessing extracorporeal membrane oxygenation circuits to perform emergent interventional cardiac catheterisation.

Cardiol Young. 2019 Oct;29(10):1290-1293

Authors: Góreczny S, McLennan D, Morgan GJ

Abstract
Cardiac catheterisation in patients on extracorporeal membrane oxygenation (ECMO) may reveal new information leading to modification of a therapeutic plan and correction of newly recognised or residual lesions. Complications associated with cardiac catheterisation during ECMO are not uncommon and often related to the access site. We report a straightforward technique for accessing the ECMO circuit to perform an emergent cardiac catheterisation in two patients with hypoplastic left heart syndrome decompensated after Norwood I, due to presumed systemic-to-pulmonary artery shunt obstruction.

PMID: 31511102 [PubMed - indexed for MEDLINE]

Related Articles

Long-term outcome of partial P450 side-chain cleavage enzyme deficiency in three brothers: the importance of early diagnosis.

Eur J Endocrinol. 2020 Mar;182(3):K15-K24

Authors: Kallali W, Gray E, Mehdi MZ, Lindsay R, Metherell LA, Buonocore F, Suntharalingham JP, Achermann JC, Donaldson M

Abstract
Objective: CYP11A1 mutations cause P450 side-chain cleavage (scc) deficiency, a rare form of congenital adrenal hyperplasia with a wide clinical spectrum. We detail the phenotype and evolution in a male sibship identified by HaloPlex targeted capture array.
Family study: The youngest of three brothers from a non-consanguineous Scottish family presented with hyperpigmentation at 3.7 years. Investigation showed grossly impaired glucocorticoid function with ACTH elevation, moderately impaired mineralocorticoid function, and normal external genitalia. The older brothers were found to be pigmented also, with glucocorticoid impairment but normal electrolytes. Linkage studies in 2002 showed that all three brothers had inherited the same critical regions of the maternal X chromosome suggesting an X-linked disorder, but analysis of NR0B1 (DAX-1, adrenal hypoplasia) and ABCD1 (adrenoleukodystrophy) were negative. In 2016, next-generation sequencing revealed compound heterozygosity for the rs6161 variant in CYP11A1 (c.940G>A, p.Glu314Lys), together with a severely disruptive frameshift mutation (c.790_802del, K264Lfs*5). The brothers were stable on hydrocortisone and fludrocortisone replacement, testicular volumes (15-20 mL), and serum testosterone levels (24.7, 33.3, and 27.2 nmol/L) were normal, but FSH (41.2 µ/L) was elevated in the proband. The latter had undergone left orchidectomy for suspected malignancy at the age of 25 years and was attending a fertility clinic for oligospermia. Initial histology was reported as showing nodular Leydig cell hyperplasia. However, histological review using CD56 staining confirmed testicular adrenal rest cell tumour (TART).
Conclusion: This kinship with partial P450scc deficiency demonstrates the importance of precise diagnosis in primary adrenal insufficiency to ensure appropriate counselling and management, particularly of TART.

PMID: 31917682 [PubMed - indexed for MEDLINE]

Related Articles

Transcatheter congenital interventions performed in low-volume non-surgical centres: Not a problem.

Arch Cardiovasc Dis. 2020 Feb 13;:

Authors: Mauran P, Akhavi A, Charbonneau A

PMID: 32063514 [PubMed - as supplied by publisher]

Related Articles

Response to Letter to 'Pregnancy and delivery outcomes from patients with repaired anomalous origin of the left coronary artery from the pulmonary artery'.

J Obstet Gynaecol Res. 2018 10;44(10):2018

Authors: Kanoh M, Inai K

PMID: 30043497 [PubMed - indexed for MEDLINE]

Percutaneous edge-to-edge repair for common atrioventricular valve regurgitation in a patient with heterotaxy syndrome, single ventricle physiology, and unbalanced atrioventricular septal defect.

Catheter Cardiovasc Interv. 2020 Feb 19;:

Authors: Tan W, Calfon Press M, Lluri G, Aboulhosn J

Abstract
Congenital heart disease patients, specifically with unbalanced atrioventricular septal defects and common atrioventricular valves requiring single ventricle palliation, have substantial morbidity and mortality. Atrioventricular valve regurgitation (AVVR) is associated with poor outcomes in single ventricle patients, and many of them require surgical treatment of AVVR in their lifetimes. We describe a unique case of transcatheter edge-to-edge valve repair using the MitraClip system (Abbott, Chicago, IL) in a single ventricle patient with severe common AVVR.

PMID: 32073720 [PubMed - as supplied by publisher]

Utility of the long DrySeal sheath in facilitating transcatheter pulmonary valve implantation with the Edwards Sapien 3 valve.

Catheter Cardiovasc Interv. 2020 Feb 19;:

Authors: Fukuda T, Tan W, Sadeghi S, Lin J, Salem M, Levi D, Aboulhosn J

Abstract
OBJECTIVE: To assess the utility of the 65-cm-long Gore DrySeal sheath when compared to the standard 36-cm-long Edwards expandable sheath (e-sheath) for transcatheter pulmonary valve implantation (TPVI) with the Edwards Sapien 3 valve.
METHODS: All patients who underwent TPVI with the Sapien 3 valve, excluding those performed via hybrid approach, at our center between September 2015 and November 2019 were retrospectively reviewed and compared between two groups.
RESULTS: A total of 94 patients were enrolled; 29 patients underwent TPVI with the Sapien valve using the DrySeal sheath and 65 underwent TPVI using the e-sheath. The height and body weight of patients implanted using the DrySeal sheath ranged from 137 to 193 cm and from 33 to 129 kg, respectively. Valve delivery time was significantly shorter in the DrySeal group (median time 4 min 33 s vs. 9 min 6 s, p = .002). There were no complications in the DrySeal group (0/27). Nine procedural complications occurred in the e-sheath group (9/65), five of which were potentially directly related to sheath choice, including tricuspid valve injury in four and embolization of the tip of the e-sheath during retrieval of a ruptured balloon in one patient.
CONCLUSIONS: TPVI with the Sapien 3 valve using the 65-cm-long DrySeal sheath facilitates faster and safer valve implantation when compared to the e-sheath.

PMID: 32073725 [PubMed - as supplied by publisher]

Related Articles

Biomechanical modeling of transcatheter aortic valve replacement in a stenotic bicuspid aortic valve: deployments and paravalvular leakage.

Med Biol Eng Comput. 2019 Oct;57(10):2129-2143

Authors: Lavon K, Marom G, Bianchi M, Halevi R, Hamdan A, Morany A, Raanani E, Bluestein D, Haj-Ali R

Abstract
Calcific aortic valve disease (CAVD) is characterized by stiffened aortic valve leaflets. Bicuspid aortic valve (BAV) is the most common congenital heart disease. Transcatheter aortic valve replacement (TAVR) is a treatment approach for CAVD where a stent with mounted bioprosthetic valve is deployed on the stenotic valve. Performing TAVR in calcified BAV patients may be associated with post-procedural complications due to the BAV asymmetrical structure. This study aims to develop refined computational models simulating the deployments of Evolut R and PRO TAVR devices in a representative calcified BAV. The paravalvular leakage (PVL) was also calculated by computational fluid dynamics simulations. Computed tomography scan of severely stenotic BAV patient was acquired. The 3D calcium deposits were generated and embedded inside a parametric model of the BAV. Deployments of the Evolut R and PRO inside the calcified BAV were simulated in five bioprosthesis leaflet orientations. The hypothesis of asymmetric and elliptic stent deployment was confirmed. Positioning the bioprosthesis commissures aligned with the native commissures yielded the lowest PVL (15.7 vs. 29.5 mL/beat). The Evolut PRO reduced the PVL in half compared with the Evolut R (15.7 vs. 28.7 mL/beat). The proposed biomechanical computational model could optimize future TAVR treatment in BAV patients. Graphical abstract.

PMID: 31372826 [PubMed - indexed for MEDLINE]

Related Articles

Case series, contemporary review and imaging guided diagnostic and management approach of congenital pericardial defects.

Open Heart. 2020;7(1):e001103

Authors: Khayata M, Alkharabsheh S, Shah NP, Verma BR, Gentry JL, Summers M, Xu B, Asher C, Klein AL

Abstract
Objective: Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition.
Methods: We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed.
Results: Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5-144.9 months) and all remained asymptomatic.
Conclusion: CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.

PMID: 32076559 [PubMed]

Related Articles

What Every Cardiologist Should Know About the 2018 Updated Adult Congenital Cardiology Guidelines.

Curr Cardiol Rep. 2020 Feb 19;22(4):24

Authors: Dolgner SJ, Buber J, Stout KK, Steinberg ZL

Abstract
PURPOSE OF REVIEW: Review the recently updated guidelines for the management of patients with adult congenital heart disease (ACHD) with a focus on the changes between these guidelines and the prior guidelines.
RECENT FINDINGS: The 2018 guidelines for the management of patients with ACHD focused on utilizing the available data and limiting the number recommendations based only on expert opinion. These guidelines implement a new anatomic and physiological classification scheme to guide management of patients, which takes into account both the underlying anatomy as well as residual cardiac disease and symptoms. Given a lack of robust outcomes data for many types of CHD, the new guidelines provide fewer total recommendations than the prior version, emphasizing the data that is available and drawing attention to the need for additional data. The 2018 guidelines provide the field with a comprehensive update in the management of ACHD patients with an emphasis on the available data.

PMID: 32076876 [PubMed - in process]

Pages