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Cardiopulmonary dysfunction in adults with a small, unrepaired ventricular septal defect: A long-term follow-up.

Int J Cardiol. 2020 Feb 27;:

Authors: Eckerström F, Rex CE, Maagaard M, Heiberg J, Rubak S, Redington A, Hjortdal VE

BACKGROUND: There are increasing reports of cardiac and exercise dysfunction in adults with small, unrepaired ventricular septal defects (VSDs). The primary aim of this study was to evaluate pulmonary function in adults with unrepaired VSDs, and secondly to assess the effects of 900 μg salbutamol on lung function and exercise capacity.
METHODS: Young adult patients with small, unrepaired VSDs and healthy age- and gender-matched controls were included in a double-blinded, randomised, cross-over study. Participants underwent static and dynamic spirometry, impulse oscillometry, multiple breath washout, diffusion capacity for carbon monoxide, and ergometer bicycle cardiopulmonary exercise test.
RESULTS: We included 30 patients with VSD (age 27 ± 6 years) and 30 controls (age 27 ± 6 years). Patients tended to have lower FEV1, 104 ± 11% of predicted, compared with healthy controls, 110 ± 14% (p = 0.069). Furthermore, the patient group had lower peak expiratory flow (PEF), 108 ± 20% predicted, compared with the control group, 118 ± 17% (p = 0.039), and showed tendencies towards lower forced vital capacity and increased airway resistance compared with controls. During exercise, the patients had lower oxygen uptake, 35 ± 8 ml/min/kg (vs 47 ± 7 ml/min/kg, p < 0.001), minute ventilation, 1.5 ± 0.5 l/min/kg (vs 2.1 ± 0.3 l/min/kg, p < 0.001) and breath rate, 48 ± 11 breaths/min (vs 55 ± 8 breaths/min, p = 0.008), than controls.
CONCLUSION: At rest, young adults with unrepaired VSDs are no different in pulmonary function from controls. However, when the cardiorespiratory system is stressed, VSD patients demonstrate significantly impaired minute ventilation and peak oxygen uptake, which may be early signs of parenchymal dysfunction and restrictive airway disease. These abnormalities were unaffected by the inhalation of salbutamol.

PMID: 32147225 [PubMed - as supplied by publisher]

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Grown-up Congenital Heart Surgery in 1093 Consecutive Cases: A 'Hidden' Burden of Early Outcome.

Ann Thorac Surg. 2020 Mar 05;:

Authors: Haapanen H, Tsang V, Kempny A, Neijenhuis R, Kennedy F, Cullen S, Walker F, Kostolny M, Hsia TY, Van Doorn C

BACKGROUND: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. While 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome.
METHODS: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day, 6-month mortality, and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into four time intervals.
RESULTS: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. Thirty-day mortality improved significantly over the study period, with an overall 30-day mortality of 1.5%. Six-month mortality and prolonged ICU stay were 2.4% and 6.7% respectively. Despite a decreased number of preoperative patients in NYHA class ≥3, prolonged ICU stay increased over the eras. Predictors of adverse outcome were; NYHA class ≥3, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures.
CONCLUSIONS: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.

PMID: 32147413 [PubMed - as supplied by publisher]

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Mechanical Circulatory Support for Patients With Adult Congenital Heart Disease.

Circ J. 2020 Mar 06;:

Authors: Haranal M, Luo S, Honjo O

Advances in surgical and medical care of children born with heart defects have led to the emergence of a unique subgroup of young adults known as adults with congenital heart disease (ACHD). Heart failure (HF) is the leading cause of mortality and morbidity in this subset. Management of HF is challenging in these patients owing to inherent structural variations with their associated physiological consequences. Heart transplantation is of limited utility in this group either because of donor shortage or associated comorbidities that make these patients ineligible for transplantation. Mechanical circulatory support (MCS) devices have evolved as an alternative treatment modality in supporting the failing myocardium of this population, but are often used less frequently than in those with a structurally normal heart because of the unique anatomical and physiological variations. These variations create a need to gather adequate knowledge on how best to support the hearts of ACHD patients in order to reduce mortality and morbidity. This review presents clinical experience with MCS in ACHD patients.

PMID: 32147603 [PubMed - as supplied by publisher]

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Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design.

ESC Heart Fail. 2020 Mar 09;:

Authors: Amedro P, Gavotto A, Abassi H, Picot MC, Matecki S, Malekzadeh-Milani S, Levy M, Ladouceur M, Ovaert C, Aldebert P, Thambo JB, Fraisse A, Humbert M, Cohen S, Baruteau AE, Karsenty C, Bonnet D, Hascoet S, SV-INHIBITION study investigators

AIMS: In univentricular hearts, selective lung vasodilators such as phosphodiesterase type 5 (PDE5) inhibitors would decrease pulmonary resistance and improve exercise tolerance. However, the level of evidence for the use of PDE5 inhibitors in patients with a single ventricle (SV) remains limited. We present the SV-INHIBITION study rationale, design, and methods.
METHODS AND RESULTS: The SV-INHIBITION trial is a nationwide multicentre, randomized, double blind, placebo-controlled, Phase III study, aiming to evaluate the efficacy of sildenafil on the ventilatory efficiency during exercise, in teenagers and adult patients (>15 years old) with an SV. Patients with a mean pulmonary arterial pressure >15 mmHg and a trans-pulmonary gradient >5 mmHg, measured by cardiac catheterization, will be eligible. The primary outcome is the variation of the VE/VCO2 slope, measured by a cardiopulmonary exercise test, between baseline and 6 months of treatment. A total of 50 patients are required to observe a decrease of 5 ± 5 points in the VE/VCO2 slope, with a power of 90% and an alpha risk of 5%. The secondary outcomes are clinical outcomes, oxygen saturation, 6 min walk test, SV function, NT-proBNP, peak VO2 , stroke volume, mean pulmonary arterial pressure, trans-pulmonary gradient, SF36 quality of life score, safety, and acceptability.
CONCLUSIONS: The SV-INHIBITION study aims to answer the question whether PDE5 inhibitors should be prescribed in patients with an SV. This trial has been built focusing on the three levels of research defined by the World Health Organization: disability (exercise tolerance), deficit (SV function), and handicap (quality of life).

PMID: 32147955 [PubMed - as supplied by publisher]

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Case 4/2019 - 26-Year-Old Man with Congenital Chagas Disease and Heart Transplantation.

Arq Bras Cardiol. 2019 09 02;113(2):286-293

Authors: Pinesi HT, Strabelli TMV, Aiello VD

PMID: 31483025 [PubMed - indexed for MEDLINE]

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Case 270: Spontaneous Coronary Artery Dissection Associated with Fibromuscular Dysplasia.

Radiology. 2019 10;293(1):235-240

Authors: Sutil-Vega M, Romeu Vilar D, Barros-Membrilla AJ, Millán X, Hidalgo JA, Pons-Lladó G

HistoryA 54-year-old woman presented with typical chest pain during physical training at the gym. She had a history of hypertension controlled with hydrochlorothiazide, without any other cardiovascular risk factor and with neither personal nor family history of ischemic heart disease. She was postmenopausal and had a long-standing history of migraine headaches without hormonal or drug therapy. The patient had no history of clinically important thoracic trauma or invasive chest interventions. Initial electrocardiography (ECG) showed signs of ongoing anterior ST segment elevation myocardial infarction, and emergent coronary angiography with angioplasty and intravascular US were performed. Maximal level of high-sensitive T troponins was 820 ng/L (normal, <13 ng/L), while echocardiography showed a normal left ventricular ejection fraction, with no apparent regional wall motion abnormalities. General physical examination findings were unremarkable, excluding ligamentous hyperlaxity and joint instability. C-reactive protein, rheumatoid factor, antinuclear antibody, cytoplasmic antineutrophil cytoplasmic antibody, and angiotensin-converting enzyme blood test results were negative. For further evaluation, arterial phase ECG-synchronized CT angiography from the skull base to the pubis symphysis was performed.

PMID: 31536471 [PubMed - indexed for MEDLINE]

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Pregnancy in non-palliated functionally single ventricle: challenges of management in resource-poor settings.

Pan Afr Med J. 2020;35:6

Authors: Delsol-Gyan D, Aniteye E, Oppong S, Ofosu-Appiah E, Edwin F

Women with complex functionally univentricular hearts rarely survive into adulthood without corrective or palliative surgery. Reports of pregnancy outcome in this group of patients in resource-poor settings are sparse. We report a case of unrepaired pulmonary atresia ventricular septal defect (VSD) with major aorto-pulmonary collateral arteries (MAPCA) who survived into adulthood and was able to complete a successful pregnancy in a resource-poor country.

PMID: 32128022 [PubMed - indexed for MEDLINE]

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Abnormal glucose metabolism in patients with Fontan circulation: Unique characteristics and associations with Fontan pathophysiology.

Am Heart J. 2019 10;216:125-135

Authors: Ohuchi H, Negishi J, Hayama Y, Miike H, Suzuki D, Nakajima K, Konagai N, Iwasa T, Sakaguchi H, Kurosaki K, Nakai M

BACKGROUND: Fontan patients exhibit a high prevalence of abnormal glucose metabolism (AGM). We aimed to characterize AGM and clarify its association with Fontan pathophysiology.
METHODS: We prospectively evaluated AGM with plasma glucose dynamics [mg/dL; fasting glucose (FPG), and maximum glucose increase (PG-spike)] during oral glucose tolerance test and hemoglobin A1c (HbA1c) in 276 consecutive Fontan patients (aged 19 ± 7 years). Of these, 176 patients had serial AGM assessments with a mean interval of 6.5 years.
RESULTS: Initial analysis revealed a high prevalence of impaired glucose tolerance (38.4%) and diabetes mellitus (DM) (4.7%), and positive family history, high HbA1c, and high central venous pressure independently predicted presence of DM. HbA1c was independently determined by hypersplenism and presence of DM (P < .05). Serial assessments revealed an increased PG-spike and a decreased HbA1c (P < .001 for both). Prevalence of DM increased (6.3% to 10.3%), and positive family history, high liver enzymes, and AGM predicted new onset of DM (P < .05 for all). Twenty-one patients died during 7.1-year follow-up. FPG (P < .01) and PG-spike (P < .05) independently predicted all-cause mortality. Particularly, patients with FPG ≤ 74 and/or PG-spike ≥85 had a mortality rate 8.7 times higher than those without (P = .0129).
CONCLUSIONS: AGM progressed even in young adult Fontan patients, and HbA1c showed limited predictive value for progression. Oral glucose tolerance test plays important roles in uncovering unique Fontan AGM as well as predicting all-cause mortality.

PMID: 31425899 [PubMed - indexed for MEDLINE]

Related Articles

Percutaneous Pulmonary Valve Implantation.

Korean Circ J. 2020 Apr;50(4):302-316

Authors: Giugno L, Faccini A, Carminati M

Percutaneous pulmonary valve implantation (PPVI) is recognized as a feasible and low risk alternative to surgery to treat dysfunctional right ventricular outflow tract (RVOT) in usually pluri-operated patients. Evolving technology allowed to develop different kind of prosthesis and to go from an initial treatment exclusively of stenotic conduit to an actual approach extended also to wide native RVOT. The Melody transcatheter pulmonary valve (TPV) and the Edwards Sapien valve are nowadays the most commonly implanted prostheses. However, other devices have been developed to treat large RVOT (i.e., the Venus p-valve, the Medtronic Harmony TPV, the Alterra Adaptive Prestent, and the Pulsta valve). Indications for PPVI are the same as for surgical interventions on pulmonary valve, with limits related to the maximum diameter of the available percutaneous prosthesis. Therefore, an accurate preoperative evaluation is of paramount importance to select patients who could benefit from this procedure. The overall periprocedural mortality incidence is around 1.4%, while freedom from RVOT reintervention ranges from 100% at 4 months to 70% at 70 months, according to the different published studies.

PMID: 32157831 [PubMed]

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Practice Variation Among Canadian Pediatric Cardiologists in Medical Management of Dilated Ascending Aorta in Patients With Bicuspid Aortic Valve.

CJC Open. 2019 May;1(3):119-122

Authors: Hussain A, Warren AE, Chen RPC, Dhillon SS

Background: Medical therapy is often prescribed to reduce the rate of aortic dilatation and prevent aortic dissection in patients with bicuspid aortic valve (BAV) despite a lack of evidence. We conducted an anonymous survey to gain insight into Canadian clinical practice regarding medical therapy used to slow the progression of aortic dilatation in patients with BAV.
Methods: A questionnaire was sent to 115 paediatric cardiologists and 18 adult congenital heart disease specialists in Canada.
Results: Ninety of 113 paediatric cardiologists (80%) completing the questionnaire reported prescribing medication to reduce the rate of aortic dilatation. Some 75% (61/81) of them reported prescribing medications on the basis of aortic size z scores, and 78% (48/61) considered medication at a z score between ≥ 2 and < 5. The remaining 25% of responders (20/81) reported prescribing medications on the basis of absolute aortic diameter, and 80% (16/20) of them considered initiating medical therapy at an aortic diameter > 40 mm to < 50 mm. For practical purposes, however, 40% of respondents (45/113) would not or rarely consider medical therapy for this indication because of variation in the threshold for initiating treatment. Ten of 14 adult congenital heart disease specialists' responses (71%), reported prescribing medications who were excluded because of missing data.
Conclusion: The majority of Canadian paediatric cardiologists reported prescribing medications to slow the rate of aortic dilatation in patients with BAV. However, there is heterogeneity in the criteria to prescribe medical therapy. A multicenter randomized controlled trial is needed to establish the role of medical therapy in this patient population.

PMID: 32159094 [PubMed]