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BACKGROUND: Patients with repaired tetralogy of Fallot (rTOF) have a time-dependent increased risk of ventricular tachycardia (VT). Slow conducting anatomical isthmuses (SCAIs) are the dominant VT substrates in adults with rTOF. It is unknown if they are present at younger age. OBJECTIVES: This study aimed to characterize VT substrates in patients with rTOF <30 years of age. METHODS: Data of consecutive patients with rTOF aged <30 years who underwent electroanatomical mapping and programmed electrical stimulation between 2005 and 2022 were analyzed. RESULTS: Fifty-five patients were included (median age: 15.8 years, IQR: 13.8-21.8 years; 15 repaired via ventriculotomy; 13 complex TOF variants). Twelve patients had right ventricle-to-pulmonary artery conduits inserted during initial repair or had early pulmonary valve replacement (PVR) (<1 year after repair). Indications for electroanatomical mapping and programmed electrical stimulation were spontaneous VT, before PVR, and risk stratification in 5, 40, and 10 patients, respectively. In 16 patients (29%), SCAI 3 was identified; no other SCAI was present. Monomorphic VT was inducible in 8 and related to SCAI 3 in 7 patients. Identified VT substrates were targeted by ablation. Right ventricle-to-pulmonary artery conduit/early PVR, ventriculotomy, and complex TOF were associated with SCAI 3 in univariable analysis. During a median follow-up of 5.3 years, VT recurred in 2 patients. No patients died. CONCLUSIONS: In young patients with rTOF, SCAI 3 is the dominant substrate for VT. Complex TOF and interrelated type and timing of (re-)operation may contribute to the development of SCAI 3 already at a young age.
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Background: Left ventricular apical pacing (LVAP) is considered to preserve left ventricular (LV) systolic function in both patients with and without congenital heart disease. However, sporadic LVAP-associated cardiac dysfunction in children with complex structural heart disease was recently reported. We present the case of a 2.5-year-old child with complex congenital heart disease and LVAP-induced cardiomyopathy. Case summary: Corrective surgery for double outlet right ventricle, subpulmonary ventricular septal defect, and transposition of the great arteries was done at the age of 1.5 months. Late complete atrioventricular block occurred, necessitating VVI pacemaker insertion with LV apical epicardial leads. He presented with heart failure and dilated cardiomyopathy 1.5 years after pacemaker insertion and required persistent circulatory support with intravenous inotropes. Speckle tracking echocardiography identified an important LV apical to basal dyssynchrony. After excluding any coronary artery involvement, cardiac resynchronization therapy was performed. Speckle tracking echocardiography guided lead placement resulted in improved LV contraction synchrony. Cardiac function recovered progressively in combination with oral heart failure medication and is almost normal at 10-month follow-up. Discussion: Right ventricular pacing is a well-known cause of pacing-induced cardiomyopathy. The LV apex and LV free wall are thought to be most optimal locations for ventricular pacing in children. However, LVAP can also be the cause of a pacing-induced cardiomyopathy and decreased systolic LV function in children with complex congenital heart disease due to lack of LV contraction synchrony. Cardiac resynchronization therapy can reverse this LV dysfunction and remodelling.
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OBJECTIVES: In patients with borderline left hearts or a severe left ventricular outflow tract obstruction, hybrid palliation can be used to stabilize the patient and postpone biventricular repair (BVR). In this study, we analysed growth of left-sided structures and outcomes of these patients. METHODS: We conducted a retrospective cohort study including patients who received hybrid palliation between January 2010 and September 2023. Echo measurements were collected at hybrid palliation, BVR and last follow-up. Growth of left ventricular structures were analysed. RESULTS: In 38 patients, hybrid palliation was used to promote growth of left ventricular structures. In total, 15 patients received a Ross-Konno/Yasui procedure, while 23 patients received conventional BVR. In patients with a conventional BVR, a significant increase was found in left ventricular volume indexed by body surface area, Z-score of aortic valve and left ventricular outflow tract between hybrid palliation and BVR. Mitral valve Z-score did not increase significantly. After BVR until follow-up, only increase of the aortic valve Z-scores and left ventricular volume indexed by body surface area was found significant. Of all included patients (n = 38), additional surgical procedures were necessary in 8 patients during the interstage period and 15 patients after BVR. Additional catheter interventions were needed in 14 patients in the interstage period and 15 after BVR. Six patients died, with no mortality in the conventional BVR group. CONCLUSIONS: Hybrid palliation as part of a staged BVR is a safe and effective initial step and promotes the growth of left ventricular structures in patients with small left-sided heart structures. Close follow-up is mandatory because extra catheter or surgical interventions are frequently needed.
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Ventrículos do Coração , Cuidados Paliativos , Obstrução do Fluxo Ventricular Externo , Humanos , Estudos Retrospectivos , Masculino , Feminino , Ventrículos do Coração/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Cuidados Paliativos/métodos , Obstrução do Fluxo Ventricular Externo/cirurgia , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem , Lactente , Procedimentos Cirúrgicos Cardíacos/métodos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Recém-Nascido , Ecocardiografia , Resultado do TratamentoRESUMO
Background: The number of patients with an arterial switch operation (ASO) for transposition of the great arteries (TGA) is steadily growing; limited information is available regarding the clinical course in the current era. Objectives: The purpose was to describe clinical outcome late after ASO in a national cohort, including survival, rates of (re-)interventions, and clinical events. Methods: A total of 1,061 TGA-ASO patients (median age 10.7 years [IQR: 2.0-18.2 years]) from a nationwide prospective registry with a median follow-up of 8.0 years (IQR: 5.4-8.8 years) were included. Using an analysis with age as the primary time scale, cumulative incidence of survival, (re)interventions, and clinical events were determined. Results: At the age of 35 years, late survival was 93% (95% CI: 88%-98%). The cumulative re-intervention rate at the right ventricular outflow tract and pulmonary branches was 36% (95% CI: 31%-41%). Other cumulative re-intervention rates at 35 years were on the left ventricular outflow tract (neo-aortic root and valve) 16% (95% CI: 10%-22%), aortic arch 9% (95% CI: 5%-13%), and coronary arteries 3% (95% CI: 1%-6%). Furthermore, 11% (95% CI: 6%-16%) of the patients required electrophysiological interventions. Clinical events, including heart failure, endocarditis, and myocardial infarction occurred in 8% (95% CI: 5%-11%). Independent risk factors for any (re-)intervention were TGA morphological subtype (Taussig-Bing double outlet right ventricle [HR: 4.9, 95% CI: 2.9-8.1]) and previous pulmonary artery banding (HR: 1.6, 95% CI: 1.0-2.2). Conclusions: TGA-ASO patients have an excellent survival. However, their clinical course is characterized by an ongoing need for (re-)interventions, especially on the right ventricular outflow tract and the left ventricular outflow tract indicating a strict lifelong surveillance, also in adulthood.
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BACKGROUND AND AIMS: Patients with repaired tetralogy of Fallot remain at risk of life-threatening ventricular tachycardia related to slow-conducting anatomical isthmuses (SCAIs). Preventive ablation of SCAI identified by invasive electroanatomical mapping is increasingly performed. This study aimed to non-invasively identify SCAI using 3D late gadolinium enhancement cardiac magnetic resonance (3D-LGE-CMR). METHODS: Consecutive tetralogy of Fallot patients who underwent right ventricular electroanatomical mapping (RV-EAM) and 3D-LGE-CMR were included. High signal intensity threshold for abnormal myocardium was determined based on direct comparison of bipolar voltages and signal intensity by co-registration of RV-EAM with 3D-LGE-CMR. The diagnostic performance of 3D-LGE-CMR to non-invasively identify SCAI was determined, validated in a second cohort, and compared with the discriminative ability of proposed risk scores. RESULTS: The derivation cohort consisted of 48 (34 ± 16 years) and the validation cohort of 53 patients (36 ± 18 years). In the derivation cohort, 78 of 107 anatomical isthmuses (AIs) identified by EAM were normal-conducting AI, 22 were SCAI, and 7 blocked AI. High signal intensity threshold was 42% of the maximal signal intensity. The sensitivity and specificity of 3D-LGE-CMR for identifying SCAI or blocked AI were 100% and 90%, respectively. In the validation cohort, 85 of 124 AIs were normal-conducting AI, 36 were SCAI, and 3 blocked AI. The sensitivity and specificity of 3D-LGE-CMR were 95% and 91%, respectively. All risk scores showed an at best modest performance to identify SCAI (area under the curve ≤ .68). CONCLUSIONS: 3D late gadolinium enhancement cardiac magnetic resonance can identify SCAI with excellent accuracy and may refine non-invasive risk stratification and patient selection for invasive EAM in tetralogy of Fallot.
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Imageamento Tridimensional , Taquicardia Ventricular , Tetralogia de Fallot , Humanos , Tetralogia de Fallot/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Masculino , Feminino , Adulto , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/diagnóstico por imagem , Adulto Jovem , Meios de Contraste , Pessoa de Meia-IdadeRESUMO
We present the case of a 52-year-old woman with cardiogenic shock and refractory right ventricular failure due to spontaneous dissection of the right coronary artery. She remained dependent on mechanical support for several weeks. Both a right ventricular assist device implant and a bidirectional cavopulmonary anastomosis were explored as long-term support options. A history of malignancy and possible right ventricular functional recovery resulted in a decision in favour of the bidirectional cavopulmonary anastomosis and concomitant tricuspid valve annuloplasty. Postoperatively her clinical condition improved significantly, and she could be discharged home. Echocardiography showed normalization of right ventricular dimensions and slight improvement of right ventricular function.
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Insuficiência Cardíaca , Infarto do Miocárdio , Humanos , Feminino , Pessoa de Meia-Idade , Insuficiência Cardíaca/cirurgia , Insuficiência Cardíaca/etiologia , Infarto do Miocárdio/cirurgia , Infarto do Miocárdio/complicações , Disfunção Ventricular Direita/cirurgia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia , Técnica de Fontan/efeitos adversos , Coração Auxiliar , Ventrículos do Coração/cirurgia , Ventrículos do Coração/diagnóstico por imagemRESUMO
BACKGROUND: Kidney disease is the most important predictor of death in patients with a Fontan circulation, yet its clinical and hemodynamic correlates have not been well established. METHODS AND RESULTS: A total of 53 ambulatory patients with a Fontan circulation (median age, 16.2 years, 52.8% male patients) underwent advanced cardiovascular magnetic resonance assessment, including 4-dimensional flow imaging and computational fluid dynamics. Estimated glomerular filtration rate (eGFR) <90 mL/min per 1.73 m2 was observed in 20.8% and albumin-to-creatinine ratio >3 mg/mmol in 39.6%. The average eGFR decline rate was -1.83 mL/min per 1.73 m2 per year (95% CI, -2.67 to -0.99; P<0.001). Lower eGFR was associated with older age, larger body surface area at examination, longer time since Fontan procedure, and lower systemic ventricular ejection fraction. Higher albumin-to-creatinine ratio was associated with absence of fenestration at the Fontan operation, and older age and lower systemic ventricular ejection fraction at the assessment. Lower cross-sectional area of the Fontan conduit indexed to flow (r=0.32, P=0.038), higher inferior vena cava-conduit velocity mismatch factor (r=-0.35, P=0.022), higher kinetic energy indexed to flow in the total cavopulmonary connection (r=-0.59, P=0.005), and higher total cavopulmonary connection resistance (r=-0.42, P=0.005 at rest; r=-0.43, P=0.004 during exercise) were all associated with lower eGFR but not with albuminuria. CONCLUSIONS: Kidney dysfunction and albuminuria are common among clinically well adolescents and young adults with a Fontan circulation. Advanced cardiovascular magnetic resonance-derived metrics indicative of declining Fontan hemodynamics are associated with eGFR and might serve as targets to improve kidney health. Albuminuria might be driven by other factors that need further investigation.
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Técnica de Fontan , Cardiopatias Congênitas , Adolescente , Adulto Jovem , Humanos , Masculino , Feminino , Creatinina , Albuminúria/etiologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Hemodinâmica , Técnica de Fontan/efeitos adversos , Técnica de Fontan/métodos , Rim , Espectroscopia de Ressonância Magnética , AlbuminasRESUMO
BACKGROUND: Balloon atrial septostomy (BAS) is an emergent and essential cardiac intervention to enhance intercirculatory mixing at atrial level in deoxygenated patients diagnosed with transposition of the great arteries (TGA) and restrictive foramen ovale. The recent recall of several BAS catheters and the changes in the European legal framework for medical devices (MDR 2017/745), has led to an overall scarcity of BAS catheters and raised questions about the use, safety, and experience of the remaining NuMED Z-5 BAS catheter. AIMS: To evaluate and describe the practice and safety of the Z-5 BAS catheter, and to compare it to the performance of other BAS catheters. METHODS: A retrospective single-center cohort encompassing all BAS procedures performed with the Z-5 BAS catheter in TGA patients between 1999 and 2022. RESULTS: A total of 182 BAS procedures were performed in 179 TGA-newborns at Day 1 (IQR 0-5) days after birth, with median weight of 3.4 (IQR 1.2-5.7) kg. The need for BAS was urgent in 90% of patients. The percentage of BAS procedures performed at bedside increased over time from 9.8% (before 2010) to 67% (2017-2022). Major complication rate was 2.2%, consisting of cerebral infarction (1.6%) and hypovolemic shock (0.5%). The rate of minor complications was 9.3%, including temporary periprocedural AV-block (3.8%), femoral vein thrombosis (2.7%), transient intracardiac thrombus (0.5%), and atrial flutter (2.2%). BAS procedures performed at bedside and in the cardiac catheterization laboratory had similar complication rates. CONCLUSIONS: BAS using the Z-5 BAS catheter is both feasible and safe at bedside and at the cardiac catheterization laboratory with minimal major complications.
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Transposição dos Grandes Vasos , Humanos , Recém-Nascido , Transposição dos Grandes Vasos/diagnóstico por imagem , Transposição dos Grandes Vasos/cirurgia , Transposição dos Grandes Vasos/complicações , Estudos Retrospectivos , Resultado do Tratamento , Catéteres , ArtériasRESUMO
Background: Anomalous aortic origin of a right coronary artery (AAORCA) with an interarterial course merits further evaluation; however, robust risk assessment strategies for myocardial ischemia and sudden cardiac death are currently lacking. The aim of this study is to explore the potential role of fractional flow reserve (FFR), instantaneous wave-free ratio (iFR), and intravascular ultrasound (IVUS) in patients with AAORCA. Methods: Consecutive adult patients with AAORCA with an interarterial course were included. Computed tomography angiography, noninvasive ischemia detection, and FFR, iFR, and IVUS were performed at baseline and during adrenaline-induced stress. External compression was evaluated with IVUS. Results: Eight patients (63% female, mean age: 53 ± 9.5 years) were included. Five patients (63%) were symptomatic, and computed tomography angiography revealed high-risk anatomy of the AAORCA in all patients. Only in 1 (12.5%) patient FFR and iFR were positive; however, this was attributed at large to concomitant diffuse atherosclerosis. In 2 of 8 (25%), IVUS revealed external compression; however, the ostial coronary surface area remained unchanged. In all patients, a conservative treatment strategy was pursued. During a mean follow-up of 29.3 months (standard deviation ±2.6 months), symptoms spontaneously disappeared in 4 of 5 (80%) and no adverse cardiac events occurred in any of the patients. Conclusions: Despite the presence of high-risk anatomy in all patients, none had proven ischemia prompting a conservative treatment strategy. No adverse cardiac events occurred during follow-up, and in the majority of patients, symptoms spontaneously disappeared. Therefore, FFR, iFR, and IVUS with pharmacologic stress merit further investigation and might contribute to ischemia-based risk stratification and management strategies in adult patients with AAORCA.
Contexte: L'anomalie de naissance de l'artère coronaire droite à partir de l'aorte (AAORCA, anomalous aortic origin of a right coronary artery) combinée à un trajet interartériel mérite un examen plus approfondi. Cependant, on observe à l'heure actuelle des lacunes en ce qui a trait à l'emploi de stratégies fiables d'évaluation du risque d'ischémie myocardique et de mort subite d'origine cardiaque. L'objectif de cette étude est d'examiner le rôle potentiel de la mesure de la réserve coronarienne (MRC), de l'évaluation du rapport instantané sans onde (iFR, instantaneous wave-free ratio) et de l'échographie intravasculaire chez des patients présentant une AAORCA. Méthodologie: Des cas de patients adultes consécutifs présentant une AAORCA combinée à un trajet interartériel ont été inclus à l'étude. Une angiographie par tomodensitométrie (TDM), une détection non invasive de la présence d'une ischémie, la MRC, l'évaluation de l'iFR et l'échographie intravasculaire ont été effectuées au début de l'étude ainsi que lors d'un stress induit par l'adrénaline. La compression externe a également été évaluée au moyen d'une échographie intravasculaire. Résultats: Huit patients (63 % de sexe féminin; âge moyen de 53 ans ± 9,5 ans) ont participé à l'étude. Cinq patients (63 %) présentaient des symptômes, et l'angiographie par TDM a révélé une AAORCA à risque élevé chez tous les patients. Les résultats de la MRC et de l'évaluation de l'iFR étaient positifs chez seulement un patient (12,5 %), ce qui est attribuable en majeure partie à une athérosclérose diffuse concomitante. Chez deux patients (25 %), l'échographie intravasculaire a montré une compression externe de l'artère coronaire droite même si l'aire de l'ostium de l'artère n'avait pas changé. Une stratégie thérapeutique prudente a été employée pour tous les patients. Pendant la période de suivi qui a duré en moyenne 29,3 mois (écart-type : ± 2,6 mois), les symptômes se sont résorbés de manière spontanée chez quatre des cinq patients (80 %), et aucun événement cardiaque indésirable n'est survenu. Conclusion: Malgré une anatomie à risque élevé chez tous les patients, aucun d'entre eux ne présentait une ischémie connue, ce qui justifiait une stratégie thérapeutique prudente. Aucun événement cardiaque indésirable n'est survenu durant la période de suivi, et les symptômes se sont résorbés de manière spontanée chez la majorité des patients. À la lumière de ces renseignements, la MRC, l'évaluation de l'iFR et l'échographie intravasculaire lors d'un stress pharmacologique devraient faire l'objet d'autres études et pourraient éventuellement être utiles dans la stratification du risque d'ischémie et dans le choix des stratégies de prise en charge des patients adultes présentant une AAORCA.
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OBJECTIVES: Aortic valve repair procedures are technically challenging, and current intraoperative evaluation methods often fail to predict the final echocardiographic result. We have developed a novel intraoperative aortic valve visualization and pressurization (AVP) device, enabling valve inspection under physiological conditions, and measuring aortic valve insufficiency (AI) during cardioplegic arrest. METHODS: The AVP device is attached to the (neo)aorta, after any type of aortic valve repair, while the heart is arrested. The root is pressurized (60-80 mmHg) using a saline solution and an endoscope is introduced. The valve is inspected, and the amount of valvular leakage is measured. Postoperative 'gold standard' transesophageal echocardiogram measurements of AI are performed and compared against regurgitation volume measured. RESULTS: In 24 patients undergoing valve-sparing root replacement, the AVP device was used. In 22 patients, postoperative echocardiographic AI was ≤ grade 1. The median leakage was 90 ml/min, IQR 60-120 ml/min. In 3 patients, additional adjustments after visual inspection was performed. In 2 patients, with complex anatomy, the valve was replaced. In one, after evaluation with the device, there was undesirable result visually and residual AI of 330 ml/min, and in another, 260 ml/min residual AI was measured and valve restriction on visual inspection. CONCLUSIONS: The novel AVP device enables intraoperative evaluation of the valve under physiological conditions, while still on arrested heart, and allows for targeted adjustments. The AVP device can be an important aid for intraoperative evaluation of the aortic valve, during valve repair and valve-sparing procedures, thereby making the operative result more predictable and the operation more efficient.
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Insuficiência da Valva Aórtica , Procedimentos Cirúrgicos Cardíacos , Humanos , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/diagnóstico por imagem , Insuficiência da Valva Aórtica/cirurgia , Aorta/cirurgia , Ecocardiografia , Resultado do TratamentoRESUMO
OBJECTIVES: In complex double outlet right ventricle (DORV) patients, the optimal surgical approach may be difficult to assess based on conventional 2-dimensional (2D) ultrasound (US) and computed tomography (CT) imaging. The aim of this study is to assess the added value of 3-dimensional (3D) printed and 3D virtual reality (3D-VR) models of the heart used for surgical planning in DORV patients, supplementary to the gold standard 2D imaging modalities. METHODS: Five patients with different DORV subtypes and high-quality CT scans were selected retrospectively. 3D prints and 3D-VR models were created. Twelve congenital cardiac surgeons and paediatric cardiologists, from 3 different hospitals, were shown 2D-CT first, after which they assessed the 3D print and 3D-VR models in random order. After each imaging method, a questionnaire was filled in on the visibility of essential structures and the surgical plan. RESULTS: Spatial relationships were generally better visualized using 3D methods (3D printing/3D-VR) than in 2D. The feasibility of ventricular septum defect patch closure could be determined best using 3D-VR reconstructions (3D-VR 92%, 3D print 66% and US/CT 46%, P < 0.01). The percentage of proposed surgical plans corresponding to the performed surgical approach was 66% for plans based on US/CT, 78% for plans based on 3D printing and 80% for plans based on 3D-VR visualization. CONCLUSIONS: This study shows that both 3D printing and 3D-VR have additional value for cardiac surgeons and cardiologists over 2D imaging, because of better visualization of spatial relationships. As a result, the proposed surgical plans based on the 3D visualizations matched the actual performed surgery to a greater extent.
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BACKGROUND: Fluid overload (FO) is known to occur frequently after pediatric cardiac surgery and is associated with morbidity and mortality. Fontan patients are at risk to develop FO due to their critical fluid balance. Furthermore, they need an adequate preload in order to maintain adequate cardiac output. This study aimed to identify FO in patients undergoing Fontan completion and the impact of FO on pediatric intensive care unit (PICU) length of stay (LOS) and cardiac events, defined as death, cardiac re-surgery or PICU re-hospitalization during follow-up. METHODS: In this retrospective single center study, the presence of FO was assessed in 43 consecutive children undergoing Fontan completion. RESULTS: Patients with more than 5% maximum FO had an extended PICU LOS (3.9 [2.9-6.9] vs. 1.9 [1.0-2.6] days; p < 0.001) and an increased length of mechanical ventilation (21 [9-121] vs. 6 [5-10] h; p = 0.001). Regression analysis demonstrated that an increase of 1% maximum FO was associated with a prolonged PICU LOS of 13% (95% CI 1.042-1.227; p = 0.004). Furthermore, patients with FO were at higher risk to develop cardiac events. CONCLUSIONS: FO is associated with short-term and long-term complications. Further studies are needed to determine the impact of FO on the outcome in this specific population.
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In the transposition of the great arteries (TGA), alterations in hemodynamics and oxygen saturation could result in fibrotic remodeling, but histological studies are scarce. We aimed to investigate fibrosis and innervation state in the full spectrum of TGA and correlate findings to clinical literature. Twenty-two human postmortem TGA hearts, including TGA without surgical correction (n = 8), after Mustard/Senning (n = 6), and arterial switch operation (ASO, n = 8), were studied. In newborn uncorrected TGA specimens (1 day-1.5 months), significantly more interstitial fibrosis (8.6% ± 3.0) was observed compared to control hearts (5.4% ± 0.8, p = 0.016). After the Mustard/Senning procedure, the amount of interstitial fibrosis was significantly higher (19.8% ± 5.1, p = 0.002), remarkably more in the subpulmonary left ventricle (LV) than in the systemic right ventricle (RV). In TGA-ASO, an increased amount of fibrosis was found in one adult specimen. The amount of innervation was diminished from 3 days after ASO (0.034% ± 0.017) compared to uncorrected TGA (0.082% ± 0.026, p = 0.036). In conclusion, in these selected postmortem TGA specimens, diffuse interstitial fibrosis was already present in newborn hearts, suggesting that altered oxygen saturations may already impact myocardial structure in the fetal phase. TGA-Mustard/Senning specimens showed diffuse myocardial fibrosis in the systemic RV and, remarkably, in the LV. Post-ASO, decreased uptake of nerve staining was observed, implicating (partial) myocardial denervation after ASO.
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OBJECTIVES: Coronary transfer remains the most crucial part of the arterial switch operation (ASO); yet, certain coronary anatomies prohibit the use of button or trap-door transfer techniques. In the rare setting of 'non-separable' single sinus coronary arteries with intramural course, the modified Yacoub aortocoronary flap technique is a viable option. The aim of this study is to describe this operative technique and review its early- and mid-term outcomes. METHODS: This retrospective analysis included all cases with 'non-separable' single sinus coronary arteries with intramural course where the modified Yacoub aortocoronary flap technique served as a bail-out option. RESULTS: Of 516 patients who underwent ASO at our institution between January 1977 and April 2022, 14 underwent the modified Yacoub aortocoronary flap technique. The median age at ASO was 10 (interquartile range 7-19) days. Hospital mortality occurred in 3 patients (21.4%), all being related to coronary complications. All hospital survivors were still alive at a median of 9.1 (interquartile range 4.2-18.3) years after the ASO. None of them developed complaints of ischaemia, ventricular arrhythmias, ventricular dysfunction or exercise intolerance. Surveillance computed tomography angiography showed stable aortocoronary relationships free from stenosis, compression and kinking. No reoperations for coronary artery problems and/or neoaortic valve or root problems were needed. CONCLUSIONS: Although close monitoring of early coronary events seems crucial to prevent perioperative mortality, the modified Yacoub aortocoronary flap technique may serve as a viable bail-out option in patients with 'non-separable' single sinus coronary anatomy with intramural course, with excellent results among hospital survivors.
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Transposição das Grandes Artérias , Doença da Artéria Coronariana , Anomalias dos Vasos Coronários , Transposição dos Grandes Vasos , Recém-Nascido , Humanos , Transposição das Grandes Artérias/efeitos adversos , Transposição das Grandes Artérias/métodos , Transposição dos Grandes Vasos/cirurgia , Estudos Retrospectivos , Doença da Artéria Coronariana/epidemiologia , Anomalias dos Vasos Coronários/cirurgiaRESUMO
AIM: To map somatic growth patterns throughout Fontan palliation and summarise evidence on its key modifiers. METHODS: Databases were searched for relevant articles published from January 2000 to December 2021. Height and weight z scores at each time point (birth, Glenn procedure, Fontan procedure and >5 years after Fontan completion) were pooled using a random effects meta-analysis. A random effects meta-regression model was fitted to model the trend in z scores over time. RESULTS: Nineteen studies fulfilled eligibility criteria, yielding a total of 2006 participants. The z scores for height and weight were markedly reduced from birth to the interstage period, but recovered by about 50% following the Glenn procedure. At >10 years after the Fontan procedure, the z scores for weight seemed to normalise despite persistent lower height, resulting in increased body mass index. The review revealed a number of modifiers of somatic growth, including aggressive nutritional management, timing of Glenn/Fontan, prompt resolution of complications and obesity prevention programmes in adolescence and adulthood. CONCLUSION: This review mapped the somatic growth of single ventricle patients and summarised key modifiers that may be amendable to improvement. These data provide guidance on strategies to further optimise somatic growth in this population and may serve as a benchmark for clinical follow-up.
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Técnica de Fontan , Cardiopatias Congênitas , Humanos , Lactente , Estudos Retrospectivos , Ventrículos do Coração/cirurgia , Técnica de Fontan/métodos , Peso Corporal , Índice de Massa Corporal , Cardiopatias Congênitas/cirurgia , Resultado do TratamentoRESUMO
In patients with critical left ventricular outflow tract obstruction but adequately sized ventricles, the treatment of choice is biventricular repair. Several options have been proposed, including neonatal Yasui or Ross-Konno operation. However, each of these procedures carries a high mortality risk, especially in syndromic neonates. Here, we report the case of a patient with 22q11.2 deletion syndrome and a diagnosis of interrupted aortic arch type B2, ventricular septal defect and left ventricular outflow tract obstruction. As a means to avoid high-risk neonatal surgery in this patient, we pursued a strategy of delayed biventricular repair involving initial hybrid Norwood palliation followed by a Yasui-type operation at 3 months. Although this strategy turned out to be successful, proactive monitoring for the development of ductal stent stenosis during follow-up after the hybrid procedure remains crucial to prevent hemodynamic complications such as cardiac failure and systemic hypoperfusion.
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OBJECTIVES: To date, it is not known if 16-20-mm extracardiac conduits are outgrown during somatic growth from childhood to adolescence. This study aims to determine total cavopulmonary connection (TCPC) haemodynamics in adolescent Fontan patients at rest and during simulated exercise and to assess the relationship between conduit size and haemodynamics. METHODS: Patient-specific, magnetic resonance imaging-based computational fluid dynamic models of the TCPC were performed in 51 extracardiac Fontan patients with 16-20-mm conduits. Power loss, pressure gradient and normalized resistance were quantified in rest and during simulated exercise. The cross-sectional area (CSA) (mean and minimum) of the vessels of the TCPC was determined and normalized for flow rate (mm2/l/min). Peak (predicted) oxygen uptake was assessed. RESULTS: The median age was 16.2 years (Q1-Q3 14.0-18.2). The normalized mean conduit CSA was 35-73% smaller compared to the inferior and superior vena cava, hepatic veins and left/right pulmonary artery (all P < 0.001). The median TCPC pressure gradient was 0.7 mmHg (Q1-Q3 0.5-0.8) and 2.0 (Q1-Q3 1.4-2.6) during rest and simulated exercise, respectively. A moderate-strong inverse non-linear relationship was present between normalized mean conduit CSA and TCPC haemodynamics in rest and exercise. TCPC pressure gradients of ≥1.0 at rest and ≥3.0 mmHg during simulated exercise were observed in patients with a conduit CSA ≤ 45 mm2/l/min and favourable haemodynamics (<1 mmHg during both rest and exercise) in conduits ≥125 mm2/l/min. Normalized TCPC resistance correlated with (predicted) peak oxygen uptake. CONCLUSIONS: Extracardiac conduits of 16-20 mm have become relatively undersized in most adolescent Fontan patients leading to suboptimal haemodynamics.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Humanos , Adolescente , Criança , Veia Cava Superior/cirurgia , Artéria Pulmonar/cirurgia , Hemodinâmica , Oxigênio , Cardiopatias Congênitas/cirurgiaRESUMO
Background: Coronary anomalies are present in one-third of all patients with transposition of the great arteries (TGA) and have been associated with increased risk of adverse outcomes after the arterial switch operation. Therefore, knowledge about coronary anatomy remains key. Case summary: A 5-day-old girl with prenatal diagnosis of Taussig-Bing anomaly (double outlet right ventricle with TGA and large subpulmonary ventricular septal defect) along with aortic arch hypoplasia and coarctation of the aorta underwent the arterial switch operation with closure of the ventricular septal defect and aortic arch repair. On preoperative echocardiography, the right (R) and left coronary artery (LCx) connected both to aortic sinus 1, suggesting 1RLCx coronary anatomy according to the Leiden Convention coronary coding system. However, intraoperative inspection led to a reclassification of the coronary anatomy: the right coronary artery and left anterior descending coronary artery connected to aortic sinus 1 (1RL) as had been observed on echocardiography, but-remarkably-the circumflex coronary artery (Cx) connected to the posterior sinus of the pulmonary trunk. As a consequence, cardioplegia was administered into both the aortic and pulmonary roots, and the circumflex coronary artery could stay in its native position without having to be transferred during the arterial switch operation. Discussion: Various disruptions during embryological development can lead to unusual coronary anatomy in TGA patients. While anomalous connection of a coronary artery to the pulmonary trunk remains exceedingly rare, care should be taken to identify this pattern when present as failure to do so may result in adverse outcomes.
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Aims: An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using computed tomography angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, proximal luminal narrowing, and an intramural segment. To date, no robust CTA criteria exist to determine the presence of an intramural segment. We aimed to deduct new CTA parameters to distinguish an intramural course of interarterial ACAOS. Methods and results: Twenty-five patients with an interarterial ACAOS (64% female, mean age 46 years, 88% right ACAOS) from two academic hospitals were evaluated. Inclusion criteria were the availability of a preoperative CTA scan (0.51â mm slice thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS [defined as 'interluminal space' (ILS)] was assessed at 2â mm intervals along the intramural segment. Analysis showed a mean ILS of 0.69 ± 0.15â mm at 2â mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27 ± 0.29â mm, P < 0.001). Interobserver agreement evaluation showed good reproducibility (intraclass correlation coefficient 0.77, P < 0.001). Receiver operator characteristic analysis demonstrated that at a cut-off ILS of <0.95â mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity. Conclusion: The ILS is introduced as a novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of <0.95â mm is indicative of an intramural segment.
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OBJECTIVES: Delayed sternal closure is a routine procedure to reduce hemodynamic and respiratory instability in pediatric patients following cardiac surgery, particularly in neonates and infants. In this setting, the possible links between sternal wound infection and delayed sternal closure are still a matter of debate. As a part of our routine, there was a low threshold for delayed sternal closure, so we reviewed our experience with sternal wound infections with a focus on potentially related perioperative risk factors, particularly delayed sternal closure. METHODS: We retrospectively identified 358 operated neonates (37%) and infants (mean age 3.6 months) in our local congenital heart disease database between January 2013 and June 2017. Potential risk factors for sternal wound infections, such as age, gender, complexity (based on Aristotle- and STS-EACTS mortality category), reoperation, use of cardiopulmonary bypass, extracorporeal membrane oxygenation, mortality and delayed sternal closure (163/358, 46%), were subjected to uni- and multivariate analysis. RESULTS: A total of 26/358 patients (7.3%) developed a superficial sternal wound infection. There were no deep sternal wound infections, no mediastinitis or sepsis. Applying univariate analysis, the prevalence of sternal wound infections was related to younger age, more complex surgery and delayed sternal closure. However, in multivariate analysis, sternal wound infection was only associated with delayed sternal closure (p = 0.013, odds ratio 8.6). Logistic regression revealed the prevalence of delayed sternal closure to be related to younger age, complexity, and the use of extracorporeal membrane oxygenation. CONCLUSION: In patients younger than one year, sternal wound infections are clearly related to delayed sternal closure. However, in our cohort, all sternal wound infections were superficial and acceptable, considering the improved postoperative hemodynamic stability.