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Anesthesia in adults with congenital heart disease.

Curr Opin Anaesthesiol. 2017 Jun;30(3):418-425

Authors: Baehner T, Ellerkmann RK

PURPOSE OF REVIEW: The current review focuses on patients with congenital heart disease (CHD) with regard to recent trends in global demographics, healthcare provision for noncardiac surgery, as well as anesthetic and perioperative care for these patients.
RECENT FINDINGS: About 40 years after milestones of surgical innovation in CHD, the number of adults with CHD (ACHD) now surpasses those of children with CHD. This development leads to the fact that even patients with complex CHD managed for noncardiac surgery are not restricted to highly specialized centers. However, preoperative risk assessment for anesthesia in these patients is complex due to underlying cardiac morbidity and substantial CHD-associated noncardiac morbidity. In addition to clinical assessment and echocardiography, biomarker measurement may be a clinically useful tool to estimate severity of heart failure in CHD patients. The high negative predictive value of NT-proBNP makes it particularly valuable as a screening tool. Further, morbidity and mortality in ACHD patients are mainly caused by arrhythmias and therefore are also relevant for perioperative management. Adverse events and perioperative death in ACHD patients in cardiac and noncardiac surgery are frequently related to intraoperative anesthetic care.
SUMMARY: Medical progress in treatment of CHD has shifted morbidity and mortality of these patients largely to adulthood. Future investigations including risk stratification of ACHD patients are necessary to further improve perioperative management, especially for low-risk and high-risk noncardiac management.

PMID: 28306681 [PubMed - indexed for MEDLINE]

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Inferior Vena Cava Thrombus in a Postpartum Patient With Fontan Physiology: A Case Report.

A A Case Rep. 2017 Sep 01;9(5):136-139

Authors: Tashjian JA, Fraint H, DiNardo J, Rouine-Rapp K

Women with complex congenital heart disease, such as those with single-ventricle physiology, are surviving into adulthood and becoming pregnant. Because of their complex physiology, common peripartum complications pose unique risks. We describe a patient with a single ventricle who underwent an external vascular conduit, nonfenestrated Fontan procedure in childhood and then presented during the postpartum period with extensive thrombosis in her lower extremity deep venous system and inferior vena cava. In this article, we will discuss single-ventricle physiology and the implications of pregnancy, anesthetic considerations, and data for maternal and fetal outcomes in this population.

PMID: 28542047 [PubMed - indexed for MEDLINE]

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Thrombotic microangiopathy in a very young infant with mitral valvuloplasty.

Pediatr Neonatol. 2018 Feb 07;:

Authors: Matsunaga Y, Ishimura M, Nagata H, Uike K, Kinjo T, Ochiai M, Yamamura K, Takada H, Tanoue Y, Hayakawa M, Matsumoto M, Hara T, Ohga S

BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear.
CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle. ADAM metallopeptidase with thrombospondin type 1 motif 13 (ADAMTS13) activity was sustained (64%) with the undetectable inhibitor. Von Willebrand factor (VWF) multimer analyses showed absent high-molecular weight multimers. Echocardiography disclosed severe mitral regurgitation. The mitral valve repair 32 days after the initial valvuloplasty led to prompt resolution of TMA. These suggested that TMA occurred in association with valvulopathy-triggered turbulent shear flow, mechanical hemolysis and endothelial damage. The consumption of large VWF multimers might account for the vascular high shear stress shown in Heyde syndrome.
CONCLUSION: The youngest case of post-operative TMA underscores the critical coagulopathy after the first surgical intervention for congenital heart disease.

PMID: 29523490 [PubMed - as supplied by publisher]

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Pregnancy in women with congenital heart disease.

BMJ. 2018 Mar 09;360:k478

Authors: Cauldwell M, Dos Santos F, Steer PJ, Swan L, Gatzoulis M, Johnson MR

PMID: 29523513 [PubMed - in process]

Red cell distribution width in adults with congenital heart disease: A worldwide available and low-cost predictor of cardiovascular events.

Int J Cardiol. 2018 Mar 02;:

Authors: Baggen VJM, van den Bosch AE, van Kimmenade RR, Eindhoven JA, Witsenburg M, Cuypers JAAE, Leebeek FWG, Boersma E, Roos-Hesselink JW

BACKGROUND: Red cell distribution width (RDW) is a standard component of the automated blood count, and is of prognostic value in heart failure and coronary heart disease. We investigated the association between RDW and cardiovascular events in patients with adult congenital heart disease (ACHD).
METHODS AND RESULTS: In this prospective cohort study, 602 consecutive patients with ACHD who routinely visited the outpatient clinic were enrolled between 2011 and 2013. RDW was measured in fresh venous blood samples at inclusion in 592 patients (median age 33 [IQR 25-41] years, 58% male, 90% NYHA I) and at four annual follow-up visits. During 4.3 [IQR 3.8-4.7] years of follow-up, the primary endpoint (death, heart failure, hospitalization, arrhythmia, thromboembolic events, cardiac intervention) occurred in 196 patients (33%). Median RDW was 13.4 (12.8-14.1)% versus 12.9 (12.5-13.4)% in patients with and without the primary endpoint (P < 0.001). RDW was significantly associated with the endpoint when adjusted for age, sex, clinical risk factors, CRP, and NT-proBNP (HR 1.20; 95% CI 1.06-1.35; P = 0.003). The C-index of the model including RDW was slightly, but significantly (P = 0.005) higher than the model without (0.74, 95% CI 0.70-0.78 versus 0.73, 95% CI 0.69-0.78). Analysis of repeated RDW measurements (n = 2449) did not show an increase in RDW prior to the occurrence of the endpoint.
CONCLUSIONS: RDW is associated with cardiovascular events in patients with ACHD, independently of age, sex, clinical risk factors, CRP, and NT-proBNP. This readily available biomarker could therefore be considered as an additive biomarker for risk stratification in these patients.

PMID: 29525069 [PubMed - as supplied by publisher]

Long-Term Follow-up of Adults Following the Atrial Switch Operation for Transposition of the Great Arteries - A Contemporary Cohort.

Heart Lung Circ. 2017 Oct 31;:

Authors: Dennis M, Kotchetkova I, Cordina R, Celermajer DS

BACKGROUND: The atrial switch operation for transposition of the Great Arteries (TGA) (Mustard or Senning Procedure) provides excellent short-term survival. Significant long-term concerns exist for these patients, however, including the ability of the right ventricle to maintain systemic perfusion and the risk of arrhythmia. We seek to describe long-term mortality and morbidity of this group of adult patients.
METHODS: Consecutive patients who had undergone an atrial switch procedure, who were aged over 16 years and who were followed up at our tertiary level adult congenital heart disease (ACHD) service in Sydney, Australia since 2000 were included. We documented mortality using a National Death Index and analysed the prospectively defined composite endpoint of "Serious Adverse Events" including death, heart failure hospitalisation and/or documented ventricular arrhythmia.
RESULTS: There were 83 patients included; mean age at most recent follow-up was 35 ± 5 years. Overall survival was 82% at 35 years and 22% of patients experienced a serious adverse event. Atrial and ventricular arrhythmias occurred in 45% and 7% of patients respectively. Eighteen (22%) patients required a pacemaker and six (7%) required implantable cardiacdefibrillator (ICD) implantation. Significant right ventricular dysfunction was present in 26% of patients on their most recent visit and this, or requirement for permanent pacing, was associated with an increased risk of serious adverse events (OR 10.22, p<0.001), (OR 4.998, p=0.04) respectively.
CONCLUSIONS: Significant mortality and morbidity accrues by mid-adult life after an atrial procedure for TGA. Right ventricular dysfunction and permanent pacing are associated with serious adverse events.

PMID: 29525133 [PubMed - as supplied by publisher]

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Complete heart block complicating the acute phase of respiratory syncytial virus bronchiolitis.

Pediatr Pulmonol. 2017 Oct;52(10):E61-E63

Authors: Karatza AA, Kiaffas M, Rammos S

Respiratory syncytial virus is the most common pathogen causing lower respiratory tract infection in infants. In recent years, the importance of extrapulmonary complications and longterm sequelae including those involving the cardiovascular system has become apparent. We report on a 10 month old infant with respiratory syncytial virus bronchiolitis, the acute phase of which was complicated by irreversible complete heart block, which was managed conservatively.

PMID: 28486756 [PubMed - indexed for MEDLINE]

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Force-Sensing Catheters During Pediatric Radiofrequency Ablation: The FEDERATION Study.

J Am Heart Assoc. 2017 May 17;6(5):

Authors: Dalal AS, Nguyen HH, Bowman T, Van Hare GF, Avari Silva JN

BACKGROUND: Based on data from studies of atrial fibrillation ablations, optimal parameters for the TactiCath (TC; St. Jude Medical, Inc) force-sensing ablation catheter are a contact force of 20 g and a force-time integral of 400 g·s for the creation of transmural lesions. We aimed to evaluate TC in pediatric and congenital heart disease patients undergoing ablation.
METHODS AND RESULTS: Comprehensive chart and case reviews were performed from June 2015 to March 2016. Of the 102 patients undergoing electrophysiology study plus ablation, 58 (57%) underwent ablation initially with a force-sensing catheter. Patients had an average age of 14 (2.4-23) years and weight of 58 (18-195) kg with 15 patients having abnormal cardiac anatomy. Electrophysiology diagnoses for the +TC group included 30 accessory pathway-mediated tachycardia, 24 atrioventricular nodal reentrant tachycardia, and 7 other. Baseline generator settings included a power of 20 W, temperature of 40°, and 6 cc/min flow during lesion creation with 11 patients (19%) having alterations to parameters. Seventeen patients (30%) converted to an alternate ablation source. A total of 516 lesions were performed using the TC with a median contact force of 6 g, force-time integral of 149 g·s, and lesion size index of 3.3. Median-term follow-up demonstrated 5 (10%) recurrences with no acute or median-term complications.
CONCLUSIONS: TactiCath can be effectively employed in the treatment of pediatric patients with congenital heart disease with lower forces than previously described in the atrial fibrillation literature. Patients with atrioventricular nodal reentrant tachycardia or atrioventricular reciprocating tachycardia may not require transmural lesions and the TC may provide surrogate markers for success during slow pathway ablation.

PMID: 28515113 [PubMed - indexed for MEDLINE]

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Liver dysfunction and congenital heart disease: Are we ready for the epidemic?

Int J Cardiol. 2017 12 15;249:169-170

Authors: Alonso-Gonzalez R

PMID: 29121721 [PubMed - indexed for MEDLINE]

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Infective endocarditis in patients with congenital heart disease: When, where and how.

Int J Cardiol. 2017 12 15;249:171-172

Authors: Montanaro C, Dimopoulos K, Shore DF

PMID: 29121722 [PubMed - indexed for MEDLINE]