journal
https://read.qxmd.com/read/38031499/cerebral-embolic-protection-during-transcatheter-stent-expansion-of-restrictive-extra-cardiac-fontan-conduit
#1
JOURNAL ARTICLE
Raymond N Haddad, Olivier Naggara, Damien Bonnet, Sophie Malekzadeh-Milani
We report a 20-year-old female patient (76 Kg/164 cm) with an extra-cardiac Fontan circulation who was referred to our institution for exertional dyspnoea and desaturation. The patient was diagnosed with a large calcified thrombus at the level of the Fontan fenestration, protruding inside the lumen of the conduit and reducing the diameter by half with a 3 mmHg pressure gradient. Transcatheter stent expansion of the obstructed extra-cardiac conduit was done with a 48 mm long XXL PTFE-covered Optimus-CVS® under temporary cerebral embolic protection with a TriGUARD-3™ deflection filter device (Keystone Heart)...
November 30, 2023: Cardiology in the Young
https://read.qxmd.com/read/38031461/neurocognitive-function-and-health-related-quality-of-life-in-adolescents-and-young-adults-with-chd-with-pulmonary-valve-dysfunction
#2
JOURNAL ARTICLE
Jeffrey D Zampi, Kimberley P Heinrich, Lisa Bergersen, Bryan H Goldstein, Sarosh P Batlivala, Stephanie Fuller, Andrew C Glatz, Michael L O'Byrne, Bradley Marino, Katherine Afton, Ray Lowery, Sunkyung Yu, Caren S Goldberg
BACKGROUND: Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood. METHODS: In this prospective longitudinal multi-institutional study, patients 13-30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention...
November 30, 2023: Cardiology in the Young
https://read.qxmd.com/read/38031460/all-that-flows-across-the-ventricular-septum-are-not-ventricular-septal-defects
#3
JOURNAL ARTICLE
Sudipta Mondal, Dinesh P Raja, Arun Gopalakrishnan
Coronary cameral fistula is a rare congenital anomaly and clinical presentation depends on the location of the defect, degree of shunting, and associated complications. We present a case of coronary cameral fistula where segmental analysis by echocardiogram helped us to avoid misdiagnosis as a ventricular septal defect.
November 30, 2023: Cardiology in the Young
https://read.qxmd.com/read/38031457/-mfap5-variant-induced-multiple-giant-thoracic-aortic-aneurysm
#4
JOURNAL ARTICLE
Yuxi Jin, Tiange Li, Shaoying Wu, Zhongqiang Liu, Yifei Li
Heritable thoracic aortic aneurysms are complex conditions characterised by the dilation or rupture of the thoracic aorta, often occurring as an autosomal-dominant disorder associated with life-threatening complications. In this case report, we present a de novo variant, MFAP5 c.236_237insA (p.N79Kfs9), which is implicated in the development of inherited thoracic aortic aneurysm. The proband, a 15-year-old male, presented with recurrent cough, dull chest pain, chest distress, vomiting, and reduced activity tolerance, leading to the diagnosis of heritable thoracic aortic aneurysms...
November 30, 2023: Cardiology in the Young
https://read.qxmd.com/read/38031446/cardiac-mri-six-months-after-the-onset-of-multisystem-inflammatory-syndrome-in-children-and-adolescents-temporally-related-to-covid-19-a-retrospective-follow-up-study
#5
JOURNAL ARTICLE
Thomas Karas, Mario Scala, Ariane Biebl, Jürgen Steiner, Franz Fellner, Gerald Tulzer, Andreas Tulzer
BACKGROUND: In 2020, reports revealed cases called multisystem inflammatory syndrome in children and adolescents temporally related to COVID-19 or multisystem inflammatory syndrome in children. A small proportion of patients suffer from persistent left ventricular dysfunction at discharge. The primary aim was to investigate if myocardial impairment persists during follow-up in these patients. METHODS: Children fulfilling the criteria for multisystem inflammatory syndrome in children with cardiac involvement hospitalized between December 2020 and February 2022 were included in this retrospective single centre study...
November 30, 2023: Cardiology in the Young
https://read.qxmd.com/read/38018154/an-extreme-case-of-cor-triatriatum-mimicking-hypoplastic-left-heart-syndrome-and-combined-pulmonary-vein-stenosis
#6
JOURNAL ARTICLE
Hee Ju Hong, Hye Won Kwon, Jae Gun Kwak, Sang Yun Lee, Yoon Seong Lee
A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins...
November 29, 2023: Cardiology in the Young
https://read.qxmd.com/read/38017672/clinical-and-genetic-characteristics-of-catecholaminergic-polymorphic-ventricular-tachycardia-combined-with-left-ventricular-non-compaction
#7
JOURNAL ARTICLE
Bihe Xu, Jing Yang, Fang Liu, Tingting Lv, Kun Li, Yifang Yuan, Siyuan Li, Yuanwei Liu, Ping Zhang
BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia is an ion channelopathy, caused by mutations in genes coding for calcium-handling proteins. It can coexist with left ventricular non-compaction. We aim to investigate the clinical and genetic characteristics of this co-phenotype. METHODS: Medical records of 24 patients diagnosed with catecholaminergic polymorphic ventricular tachycardia in two Chinese hospitals between September, 2005, and January, 2020, were retrospectively reviewed...
November 29, 2023: Cardiology in the Young
https://read.qxmd.com/read/38014584/parental-holding-of-infants-improves-haemodynamics-in-the-cardiac-icu
#8
JOURNAL ARTICLE
Justin J Elhoff, Sebastian Acosta, Saul Flores, Julie LaSalle, Rohit Loomba, Molly McGetrick, Christy McKinney, Megan Ostrom, Chetna K Pande, Robin Schlosser, Sarah Schwab, Fabio Savorgnan
We performed a single-centre, retrospective study to assess physiologic changes of infants in the cardiac ICU while being held by their parent. Continuous data streaming of vital signs were collected for infants included in the study from January 2021 to March 2022. Demographic and clinical characteristics were collected from the electronic medical record. The physiologic streaming data were analysed using mixed-effects models to account for repeated measures and quantify the effect of parental holding. Comparison analysis was also performed controlling for intubation, pre-operative versus post-operative status, and whether the holding was skin-to-skin or not...
November 28, 2023: Cardiology in the Young
https://read.qxmd.com/read/38014551/outcomes-after-initial-heart-failure-consultation-in-fontan-patients
#9
JOURNAL ARTICLE
Sharon Chen, Muhammad F Shezad, Angela Lorts, Amanda D McCormick, Chad Y Mao, Kathleen E Simpson, Matthew J O'Connor, Aliessa Barnes, Adam M Lubert, Chesney Castleberry, Julie Schmidt, Katie Schroeder, Anna Joong, David W Bearl, Ashwin K Lal, Deepa Mokshagundam, Jennifer Conway, Ari Cedars, Kurt R Schumacher
BACKGROUND: Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care. METHODS: This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care. Part 1 of the survey captured data on clinical characteristics at the time of heart failure consultation, and Part 2, completed 30 days later, captured outcomes (death, transplant evaluation outcome, and other interventions)...
November 28, 2023: Cardiology in the Young
https://read.qxmd.com/read/38014533/the-family-burden-of-paediatric-heart-disease-during-the-chronic-phase-of-illness
#10
JOURNAL ARTICLE
Kevin Hummel, Avital Ludomirsky, Lauren Burgunder, Minmin Lu, Sarah Goldberg, Lynn Sleeper, Jeffrey Reichman, Elizabeth D Blume
BACKGROUND: CHD is a lifelong condition with a significant burden of disease to patients and families. With increased survival, attention has shifted to longer-term outcomes, with a focus on social determinants of health. Among children with CHD, socioeconomic status is associated with disparities in outcomes. Household material hardship is a concrete measure of poverty and may serve as an intervenable measure of socioeconomic status. METHODS: A longitudinal survey study was conducted at multiple time points (at acute hospitalisation, then 12-24 months later in the chronic phase) to determine the prevalence of household material hardship among parents of children with advanced heart disease and quality of life during long-term follow-up...
November 28, 2023: Cardiology in the Young
https://read.qxmd.com/read/38014532/the-neonate-score-predicts-freedom-from-interstage-mortality-or-transplant-in-a-modern-cohort
#11
JOURNAL ARTICLE
Humera Ahmed, Jeffrey B Anderson, Katherine E Bates, Carole M Lannon, David W Brown
BACKGROUND: Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation. OBJECTIVES: We sought to validate the score in an external, modern cohort. METHODS: This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation...
November 28, 2023: Cardiology in the Young
https://read.qxmd.com/read/37990583/paediatric-hypertrophic-cardiomyopathy-secondary-to-danon-disease
#12
JOURNAL ARTICLE
Kai Tang, Jiayi Wu, Xiaoxi Liu, Shuiyun Wang
Danon disease is a rare X-linked disorder caused by deficiency of the lysosome-associated membrane protein-2. We report a case of hypertrophic obstructive cardiomyopathy secondary to a novel mutation in the lysosome-associated membrane protein-2 gene in a 10-year-old male adolescent. We performed a modified extended Morrow procedure to minimise the risk of death and improve the patient's quality of life. The patient did not have exertional dyspnoea, and auscultation did not reveal a cardiac murmur at 1-year follow-up...
November 22, 2023: Cardiology in the Young
https://read.qxmd.com/read/37981897/are-dynamic-measurements-of-central-venous-pressure-in-fontan-circulation-during-exercise-or-volume-loading-superior-to-resting-measurements
#13
JOURNAL ARTICLE
Alyssia Venna, Shriprasad Deshpande, Tacy Downing, Anitha John, Yves d'Udekem
BACKGROUND: The main objective measure to assess the health of the Fontan circulation is the pressure measurement of the superior vena cava or pulmonary arteries. We reviewed the literature for benefits of measuring resting pressure in the Fontan circuit and explored whether dynamic measurement by volume loading or exercise has the potential to refine this diagnostic tool. METHODS: PubMed was searched for articles showing a relationship between resting post-operative central venous pressure or pulmonary artery pressure and Fontan failure...
November 20, 2023: Cardiology in the Young
https://read.qxmd.com/read/37981864/extubation-in-operating-room-versus-early-extubation-in-icu-after-open-heart-surgery-in-patients-with-chds
#14
JOURNAL ARTICLE
Chan Hyeong Kim, Jae Hong Lee, Hye Won Kwon, Sungkyu Cho, Woong-Han Kim, Sang-Hwan Ji, Young-Eun Jang, Jin-Tae Kim, Jae Gun Kwak
BACKGROUNDS AND OBJECTIVES: The "Fast track" protocol is an early extubation strategy to reduce ventilator-associated complications and induce early recovery after open-heart surgery. This study compared clinical outcomes between operating room extubation and ICU extubation after open-heart surgery in patients with CHD. METHODS: We retrospectively reviewed 215 patients who underwent open-heart surgery for CHDs under the scheduled "Fast track" protocol between September 2016 and April 2022...
November 20, 2023: Cardiology in the Young
https://read.qxmd.com/read/37968238/left-ventricle-hypertrophy-and-re-modeling-in-children-with-essential-hypertension-does-the-race-matter
#15
JOURNAL ARTICLE
Daiji Takajo, Thomas S Przybycien, Preetha L Balakrishnan, Girija Natarajan, Gautam K Singh, Sanjeev Aggarwal
BACKGROUND: This is the first study to report on the impact of race on differences in the prevalence of echocardiographic left ventricular hypertrophy and left ventricular adaptation at the time of diagnosis of essential hypertension in children. METHODS: This cross-sectional, single-centre study included patients aged 3-18 years who had newly diagnosed essential hypertension. Echocardiography was used to assess left ventricular mass index and left ventricular relative wall thickness...
November 16, 2023: Cardiology in the Young
https://read.qxmd.com/read/37965721/perception-towards-palliative-care-among-patients-with-pulmonary-hypertension-in-malaysia-a-correlation-with-disease-status
#16
JOURNAL ARTICLE
Sue Kiat Tye, Norazlina Susila Razali, Shakila Amira Ahmad Shauqi, Nor Athirah Azeman, Nurul Fazira Basran, Janet Huey Jing Liew, Ming Chern Leong
OBJECTIVES: This study aimed to describe the perception of Malaysian patients with pulmonary hypertension towards palliative care and their receptivity towards palliative care. METHODS: This was a cross-sectional, single-centre study conducted via questionnaire. Patients aged 18 years old and above, who were diagnosed with non-curable pulmonary hypertension were recruited and given the assessment tool - perceptions of palliative care instrument electronically. The severity of pulmonary hypertension was measured using WHO class, N-terminal pro B-type natriuretic peptide and the 6-minute walking test distance...
November 15, 2023: Cardiology in the Young
https://read.qxmd.com/read/37965690/neurodevelopment-outcomes-in-the-first-5-years-of-the-life-of-children-with-transposition-of-the-great-arteries-surgically-corrected-in-the-neonatal-period-systematic-review-and-meta-analysis
#17
REVIEW
Catarina Soares, Rafael José Vieira, Sandra Costa, Rita Moita, Mariana Andrade, Hercília Guimarães
OBJECTIVES: In patients with transposition of the great arteries, surgical correction may achieve definitive treatment, so a thorough knowledge of the long-term outcomes, particularly neurodevelopment outcomes, is essential. Therefore, we conducted a systematic review and meta-analysis to study the neurodevelopment outcomes in the first 5 years of the life of children submitted to corrective surgery for transposition of the great arteries in the neonatal period. METHODS: A total of 17 studies from 18 reports were included, assessing 809 individuals with surgically corrected transposition of the great arteries...
November 15, 2023: Cardiology in the Young
https://read.qxmd.com/read/37955043/the-mid-term-outcomes-of-aortic-root-replacement-after-surgical-repair-for-chd
#18
JOURNAL ARTICLE
Akihisa Furuta, Takeshi Shinkawa, Saeko Yoshizawa, Satoshi Okugi, Hiroshi Niinami
OBJECTIVE: The purpose of this study is to assess the mid-term outcomes of aortic root replacement after repair of CHDs. METHOD: This is a single-institutional retrospective, cohort study with consecutive patients undergoing aortic root replacement after surgical repair of CHDs between 1999 and 2022. Operative indications included aortic root dilatation with/without aortic insufficiency, sinus of Valsalva rupture, or aortic dissection involving the root. RESULTS: Forty-four patients (36 male and 8 female) were enrolled...
November 13, 2023: Cardiology in the Young
https://read.qxmd.com/read/37953639/submitral-aneurysm-correction-with-mitral-valve-repair-a-case-report
#19
JOURNAL ARTICLE
Valdano Manuel, Marília da Costa, Joseneyde Carvalho
Submitral aneurysm is a rare type of non-ischaemic ventricular aneurysm. We describe the case of a 26-year-old woman with progressive dyspnoea for approximately 1 year who was diagnosed with a ruptured submitral aneurysm in the left atrium with severe regurgitation. The patient underwent aneurysm correction and mitral valve repair, and post-operative echocardiography showed no residual abnormalities. The patient remains asymptomatic.
November 13, 2023: Cardiology in the Young
https://read.qxmd.com/read/37946577/a-rare-combination-a-single-common-pulmonary-vein-with-polysplenia-syndrome
#20
JOURNAL ARTICLE
Meryem Beyazal, Seçil Sayın, Havva Akmaz Unlu, Yasemin Özdemir Şahan
A single common pulmonary vein is a rare anomaly, mostly occurring with asplenia. We wanted to report our case because it co-exists with polysplenia and ciliary dyskinesia.A baby girl about 2 months old with respiratory arrest was admitted to the emergency room of our hospital. She responded to pulmonary resuscitation. We ascertained from her medical history that she had been hospitalised twice because of recurrent lung infections. After the initial check and treatment, we applied imaging modalities to detect underlying problems of recurrent lung infection...
November 10, 2023: Cardiology in the Young
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