Journal Watch

The Need for Extracorporeal Membrane Oxygenation in Adults Undergoing Congenital Heart Surgery: Impact and Trends of Utilization.

Indian J Crit Care Med. 2017 Sep;21(9):547-551

Authors: Aiello S, Loomba RS, Kriz C, Buelow M, Aggarwal S, Arora RR

INTRODUCTION: Adults with congenital heart disease (ACHD) represent a population with unique health-care needs. Many patients require cardiac surgery, with some requiring postoperative extracorporeal membrane oxygenation (ECMO). This study aimed to identify the risk factors for the need of postoperative ECMO and characterize the impact of ECMO on admission characteristics.
METHODS: Data from the 2005-2012 iterations of the Nationwide Inpatient Sample were used. ACHD admissions over 18 years with a documented cardiac surgery were included. Univariate analysis was conducted to compare the characteristics between those requiring ECMO and those who did not. Regression analysis was done to identify the independent risk factors associated with ECMO and to determine the impact of ECMO on length, cost, and mortality of the admission.
RESULTS: A total of 186,829 admissions were included. Of these, 446 (0.2%) admissions required ECMO. Those with acute kidney injury, double-outlet right ventricle, or total anomalous pulmonary venous connection were more likely to require ECMO. ECMO was also significantly more utilized in patients undergoing septal defect repair, complete repair of tetralogy of Fallot, atrial switch, and heart transplant. The use of ECMO significantly increased length, cost, and mortality of stay. Overall mortality was 62.6% in the ECMO group.
CONCLUSION: ECMO is only needed in a small proportion of postoperative ACHD patients. The use of ECMO significantly increases cost, length of stay and mortality in these patients. Improved identification of postoperative ACHD patients who are more likely to survive ECMO may facilitate improved survival and decreased resource utilization.

PMID: 28970652 [PubMed]

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation.

Congenit Heart Dis. 2017 Oct 02;:

Authors: Baker-Smith CM, Carlson K, Ettedgui J, Tsuda T, Jayakumar KA, Park M, Tede N, Uzark K, Fleishman C, Connuck D, Likes M, Penny DJ

OBJECTIVE: To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).
DESIGN: Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an "open comment period." Final approval of each QM was provided by a vote of the ACC ACPC Council.
PATIENTS: Patients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded.
RESULTS: Twelve candidate QMs were generated. Seven metrics passed the RAND-UCLA process. Four passed the "open comment period" and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age.
CONCLUSIONS: Application of the RAND-UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.

PMID: 28971577 [PubMed - as supplied by publisher]

Aryl Hydrocarbon Receptor Ablation in Cardiomyocytes Protects Male Mice from Heart Dysfunction Induced by NKX2.5 Haploinsufficiency.

Toxicol Sci. 2017 Aug 14;:

Authors: Wang Q, Fan Y, Kurita H, Jiang M, Koch S, Rao MB, Rubinstein J, Puga A

Epidemiological studies in humans and research in vertebrates indicates that developmental exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD), a ubiquitous and biopersistent environmental toxicant, is associated with incidence of early congenital heart disease in the embryo and later in the adult. TCDD-mediated toxicity depends on the aryl hydrocarbon receptor (AHR) but the role of the TCDD-activated AHR in cardiac function is not well defined. To characterize the mechanisms responsible for AHR-mediated disruption of heart function, we generated several mouse strains with cardiomyocyte-specific Ahr gene knockout. Here we report results on one of these strains in which the Ahr gene was deleted by cre recombinase regulated by the promoter of the cardiomyocyte-specific Nkx2.5 gene. We crossed mice with loxP-targeted Ahrfx/fx alleles with Nkx2.5+/cre mice bearing a "knock-in" cre recombinase gene integrated into one of the Nkx2.5 alleles. In these mice, loss of one Nkx2.5 allele is associated with disrupted cardiac development. In males, Nkx2.5 hemizygosity resulted in cardiac haploinsufficiency characterized by hypertrophy, dilated cardiomyopathy and impaired ejection fraction. Ahr ablation protected Nkx2.5+/cre haploinsufficient males from cardiac dysfunction while inducing a significant increase in body weight. These effects were absent or largely blunted in females. Starting at three months of age, mice were exposed by oral gavage to 1μg/kg/week of TCDD or control vehicle for an additional two months. TCDD exposure restored cardiac physiology in aging males, appearing to compensate for the heart dysfunction caused by Nkx2.5 hemizygosity. Our findings underscore the conclusion that deletion of the Ahr gene in cardiomyocytes protects males from heart dysfunction due to NKX2.5 haploinsufficiency.

PMID: 28973413 [PubMed - as supplied by publisher]

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Prenatal diagnosis of idiopathic infantile arterial calcification without fetal hydrops.

Echocardiography. 2017 Feb;34(2):311-314

Authors: Yi Y, Tong T, Liu T, Lin Q, Xiong Y, Xu J

Idiopathic infantile arterial calcification (IIAC) is a rare autosomal recessive disease that is characterized by extensive calcification of the internal elastic lamina and intimal proliferation of large- and medium-sized arteries, including the aortic, coronary, pulmonary, and iliac arteries. Most reported cases of IIAC were diagnosed in the neonatal periods. Prenatal diagnosis of this condition is extremely rare and is usually made in the third trimester when fetuses had nonimmune hydrops together with aortic and pulmonary calcification. Early prenatal diagnosis can hardly be made without fetal hydrops in the second trimester. We report a case of IIAC referred to our center because of hyperechogenic tricuspid valve. The prenatal diagnosis was made by echocardiographic detection of diffuse hyperechogenicity of the cardiac valves, annuli, aorta, pulmonary artery, renal artery and common iliac artery without fetal hydrops. To the best of our knowledge, this was the first case of IIAC accurately diagnosed prenatally in the absence of fetal hydrops.

PMID: 27928837 [PubMed - indexed for MEDLINE]

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Natural history of nonimmune-mediated thrombocytopenia and acute kidney injury in pediatric open-heart surgery.

Paediatr Anaesth. 2017 Mar;27(3):305-313

Authors: Tew S, Fontes ML, Greene NH, Kertai MD, Ofori-Amanfo G, Jaquiss RD, Lodge AJ, Ames WA, Homi HM, Machovec KA, Jooste EH

OBJECTIVE: Thrombocytopenia and acute kidney injury (AKI) are common following pediatric cardiac surgery with cardiopulmonary bypass (CPB). However, the relationship between postoperative nadir platelet counts and AKI has not been investigated in the pediatric population. Our objective was to investigate this relationship and examine independent predictors of AKI.
DESIGN: After IRB approval, we performed a retrospective review of the institution's medical records and database.
SETTING: This study was performed at a single institution over a 5-year period.
PATIENTS: We included patients <21 years of age undergoing cardiac surgery with CPB.
INTERVENTIONS: Demographics, laboratory, and surgical characteristics were captured, and clinical event rates were recorded.
MEASUREMENTS: Descriptive statistics were used to evaluate platelet and creatinine distributions. T-tests and chi-squared tests were used to compare characteristics among Acute Kidney Injury Network groups. Multivariable logistic and ordinal logistic regression models were used to determine the association of our predictor of interest, postoperative nadir platelet count and AKI.
RESULTS: Eight hundred and fourteen patients (23% infants and 23% neonates) were included in the analysis. Postoperative platelet counts decreased 48% from baseline reaching a mean nadir value of 150 × 10(9) ·l(-1) on postoperative day 3. AKI occurred in 37% of patients including 13%, 17%, and 6% with Acute Kidney Injury Network stages 1, 2, and 3, respectively. The magnitude of nadir platelet counts correlated with the severity of AKI. Independent predictors of severity of AKI include nadir platelet counts, CPB time, Aristotle score, patient weight, intra-operative packed red blood cell transfusion, and having a heart transplant procedure.
CONCLUSIONS: In pediatric open-heart surgery, thrombocytopenia and AKI occur commonly following CPB. Our findings show a strong association between nadir platelet counts and the severity of AKI.

PMID: 28098429 [PubMed - indexed for MEDLINE]

Challenges of congenital heart disease in grown-up patients.

Swiss Med Wkly. 2017 Oct 04;147:w14495

Authors: Schwerzmann M, Schwitz F, Thomet C, Kadner A, Pfammatter JP, Wustmann K

Nowadays, more than 90% of all children born with congenital heart disease (CHD) reach adult life. Although initially considered to be cured, the majority of them continue to need specialised follow-up because they require re-do interventions or are at increased risk of cardiovascular complications and premature death. Arrhythmias are the most common cause of unscheduled hospital visits for grown-up CHD (GUCH) patients, accounting for one third of emergency admissions in these patients. Some GUCH patients are also at increased risk for sudden cardiac death. The principles of arrhythmia management and the prevention of sudden cardiac death in GUCH patients are similar to those used in adults with acquired heart disease, but are not evidence based. Decompensated heart failure is the other leading cause of death. Conventional medical heart-failure therapy for left ventricular dysfunction is not effective in GUCH patients at highest risk of heart failure, i.e., those with right or single ventricular failure. Careful haemodynamic assessment and structural interventions are the first step to consider in GUCH patients presenting with heart failure symptoms. Adults with moderate or complex CHD and regular follow-up in specialised GUCH centres have a survival benefit compared with patients without such follow-up. Cardiac surgery in GUCH patients should be performed by surgeons trained in treatment of CHD, i.e., surgeons also operating on paediatric patients. A structured transition programme with a defined transfer of care from the paediatric to the adult care environment is important to avoid lapses of care in today's adolescents with CHD. For GUCH patients with an intervention performed decades ago and no specific cardiac follow-up in later life, referral to a specialised GUCH centre is recommended and may save lives.

PMID: 28975959 [PubMed - in process]

False Positive Perfusion/Ventilation SPECT Study for Pulmonary Embolism in a Patient with Fontan Circulation.

Mol Imaging Radionucl Ther. 2017 Oct 03;26(3):131-134

Authors: Panagiotidis E

Fontan circulation is the consequence of an operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. An 18-year old man with a history of congenital heart disease surgically treated with Fontan circulation, presented with pleuritic chest pain and a raised D-dimer level. Perfusion/ventilation SPECT was performed to exclude the possibility of pulmonary embolism (PE) that showed unilateral reduced perfusion of the left lung with a mismatched right upper lobe defect, suspicious of PE. However, subsequent computed tomography pulmonary angiogram and clinical follow-up excluded the possibility of PE, emphasizing the need for knowledge of potential pitfalls to avoid false interpretations. Given the fact that adult congenital heart disease population is growing, with the majority having single ventricle/Fontan circulation and being at risk for thromboembolic disease, knowledge of the perfusion pattern pitfalls is important to avoid false interpretation and preventing the misdiagnosis of PE in patients with Fontan physiology.

PMID: 28976338 [PubMed]

Repair of Double Orifice Left AV Valve (DOLAVV) with Endocardial Cushion Defect in Adult.

Braz J Cardiovasc Surg. 2017 Jul-Aug;32(4):338-340

Authors: Pillai VV, Karunakaran J

Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.

PMID: 28977206 [PubMed - in process]

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Lone Pediatric Atrial Fibrillation in the United States: Analysis of Over 1500 Cases.

Pediatr Cardiol. 2017 Jun;38(5):1004-1009

Authors: El-Assaad I, Al-Kindi SG, Saarel EV, Aziz PF

Little is known about lone atrial fibrillation (AF) in pediatrics and its risk factors due to low prevalence. We sought to determine risk factors and estimate recurrence rates in children with lone AF using a large clinical database. Using the Explorys clinical database, we retrospectively identified patients who were below 20 years of age at the time of their AF diagnosis. Patients with congenital heart disease, cardiomyopathy, prior open heart surgery, or thyroid disease were excluded. Out of 7,969,230 children identified, 1910 had AF and 1570 met the definition of lone AF. The prevalence of lone AF was 7.5 per 100,000 children. In comparison to young children (0-4 years), risk for lone AF increased with age (adjusted odds ratio (aOR) 1.2 [95% CI 0.9-1.5, P = 0.21] in those 5-9 years, aOR 1.7 [95% CI 1.3-2.1, P < 0.001] in those 10-14 years, and aOR 10.7 [95% CI 8.7-13.2, P < 0.001] in those 15-19 years). Risk of lone AF was also higher in males than females (aOR 1.7 [95% CI 1.5-1.9, P < 0.001]), and was higher in obese children (BMI ≥ 95th percentile) versus children with normal BMI (aOR 1.3 [95% CI 1.1-1.5], P < 0.001), but there was no difference between overweight (BMI = 85th-94th percentile) and normal (P = 0.14). One-month recurrence rate was 15%, and increased with age. In this large pediatric cohort, the prevalence of lone AF was low, but risk was higher in males and increased with age and obesity. Older children with lone AF had higher rates of recurrence.

PMID: 28374048 [PubMed - indexed for MEDLINE]

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Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients.

Pediatr Cardiol. 2017 Jun;38(5):981-990

Authors: Turquetto ALR, Canêo LF, Agostinho DR, Oliveira PA, Lopes MICS, Trevizan PF, Fernandes FLA, Binotto MA, Liberato G, Tavares GMP, Neirotti RA, Jatene MB

Central factors negatively affect the functional capacity of Fontan patients (FP), but "non-cardiac" factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8-17) years, respectively. Dominant ventricle ejection fraction was within normal range. The mean of peak VO2 expressed in absolute values (L/min), the relative values to body weight (mL/kg/min), and their predicted values were lower in FP compared with HC: 1.69 (±0.56) vs 2.81 (±0.77) L/min; 29.9 (±6.1) vs 41.5 (±9.3) mL/kg/min p < 0.001 and predicted VO2 Peak [71% (±14) vs 100% (±20) p < 0.001]. The absolute and predicted values of the forced vital capacity (FVC), forced expiratory volume in one second (FEV1), inspiratory capacity (IC), total lung capacity (TLC), diffusion capacity of carbon monoxide of the lung (DLCO), maximum inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) were also significantly lower in the Fontan population compared to HC. An increased risk of restrictive ventilatory pattern was found in patients with postural deviations (OD:10.0, IC:1.02-97.5, p = 0.042). There was a strong correlation between pulmonary function and absolute peak VO2 [FVC (r = 0.86, p < 0.001); FEV1 (r = 0.83, p < 0.001); IC (r = 0.84, p < 0.001); TLC (r = 0.79, p < 0.001); and DLCO (r = 0.72, p < 0.001). The strength of the inspiratory muscles in absolute and predicted values was also reduced in FP [-79(±28) vs -109(±44) cmH2O (p = 0.004) and 67(±26) vs 89(±36) % (p = 0.016)]. Thus, we concluded that the pulmonary function was impaired in clinically stable Fontan patients and the static and dynamic lung volumes were significantly reduced compared with HC. We also demonstrated a strong correlation between absolute Peak VO2 with the FVC, FEV1, TLC, and DLCO measured by complete pulmonary test.

PMID: 28500413 [PubMed - indexed for MEDLINE]