August

Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation.

Adult Congenital Heart Reviews Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation. Ono M, Burri M, Mayr B, Anderl L, Strbad M, Cleuziou J, Hager A, Hörer J, Lange R. Ann Thorac Surg. 2020 Aug 20:S0003-4975(20)31323-0. doi: 10.1016/j.athoracsur.2020.06.030. Online ahead of print. PMID: 32828751 Take Home Points: The outcome of Glenn shunt is generally good with the majority of the patients proceeding to Fontan completion. Hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure and a reduced ventricular function at the time of superior cavopulmonary shunt were identified as risk factors for failure to successfully complete the Fontan procedure. Atrio-ventricular valve regurgitation, despite associated with unbalanced atrioventricular septal defect and a reduced ventricular function, is not associated with failure to successful Fontan completion. Commentary from Dr. MC Leong (Kuala Lumpur, Malaysia), section editor of ACHD Journal Watch: While the outcomes of total cavopulmonary connections are widely reported, there is a paucity of outcome data on the second stage single ventricular palliation. Such palliation includes fashioning of a superior cavopulmonary connection (bidirectional Glenn shunt) or in some centers, a hemi-Fontan procedure. This study aimed at analyzing the outcomes of all single ventricles which have undergone the second stage palliation and identifying the factors that are associated with death or failure to progress to a Fontan completion. This is a single center, retrospective review of a database of 525 patients with single ventricles who underwent bidirectional Glenn shunt at the German Heart Center in Munich between January 1998 and December 2018. Baseline characteristics of the patient population were as tabulated in Table 1. The median time of Glenn shunt was 4.7 month (IQR, 3.0-7.4) while the median weight was 5.6 kg (IQR, 4.7-7.0). The perioperative data was summarized in Table 2. Of note is the patients with unbalanced atrioventricular septal defect were at greatest risk of early death (10.0%), whereas early death occurred in hypoplastic left heart syndrome patients was only 3.6% of all patients. Interestingly, in the early postoperative period, 55 patients required cardiac catheterization because of severe cyanosis and subsequent surgical/catheter interventions. The etiology for cyanosis were Glenn pathway stenosis (n=31), veno-venous collaterals (n=11), cavopulmonary pathway thrombus (n=4), and undetermined (n=9). In all 9 patients whose etiology was undetermined, placement of a systemic to the branch pulmonary shunt and performing a pulmonary septation to create unilateral Glenn shunt to the contralateral pulmonary artery was performed, out of which, five patients died postoperatively.   Of the 514 early survivors, 15 patients were lost to follow-up after hospital discharge (Figure 1). The median follow-up period was 3.4 years (IQR, 1.5-8.7) for the remaining 499 patients. A competitive risk analysis was performed onto patients who had Fontan completion, death, and 'being alive without Fontan'. Patients who failed to achieve successful Fontan completion was defined as those who died before Fontan completion; patients who were considered unsuitable for Fontan completion; and patients who died early after Fontan completion (death within 30 days), because they failed to establish successful Fontan circulation. The cumulative incidence of Fontan completion (83.9% at 3 years and 87.1% at 5 years), the cumulative incidence of death (10.4% at 3 years and 10.7% at 5 years), and survival without Fontan completion (5.7% at 3 years and 2.2% at 5 years) are shown in Figure 2. Freedom from mortality before the Fontan procedure, unsuitability for the Fontan procedure, and early mortality after the Fontan procedure at 1, 2, and 3 years were 91.9%, 87.3%, and 86.1%, respectively. Figure 1. Figure 2. Multivariate analysis showed that hypoplastic left heart syndrome, unbalanced atrio-ventricular septal defect (AVSD), high pulmonary artery pressure and a reduced ventricular function at Glenn shunt were associated with failure to achieve an eventual successful Fontan completion. Although significant atrioventricular valve regurgitation requiring intervention was not identified as an independent risk in multivariate model, the incidence of atrioventricular valve intervention was significantly higher in patients with unbalanced AVSD (26.7% vs. 10.3%, p=0.006) and in patients with reduced ventricular function (26.1% vs. 9.8%, p=0.001). Apart from the conclusions that the study came to, this study also raised two important issues. (1) The timing of Glenn shunt needs to be balanced between overloading the ventricle with a systemic to pulmonary artery shunt and the need to grow the pulmonary artery to a caliber suitable for the eventual Fontan completion. In this study, the Glenn shunts were performed relatively early at the median age of 4.7 month (IQR, 3.0-7.4). Meanwhile, pulmonary artery reconstruction was required in 189 (36%) patients during Glenn shunt. As many as 129 (25%) patients required bilateral branch pulmonary arteries repair. It is possible that many of them required pulmonary artery intervention to enlarge the pulmonary arteries, which may alter the dimension of the pulmonary artery outside of the lungs but not the pulmonary arterial tree within the lungs. The calibers of the pulmonary arteries are not only anatomical but also physiologically linked to the long-term outcome of the eventual Fontan procedure; (2) If a patient presents with unexplained cyanosis post Glenn shunt, the prognosis is poor. In this study, 9 patients had no recognizable anatomic defect, and hypoxemia was perceived to be secondary to inadequate pulmonary blood flow. Implanting an additional systemic to pulmonary shunt and pulmonary artery septation which supposedly meant to improve oxygenation, unfortunately, yielded in 5 deaths. These patients may perhaps benefit from other treatment options.  

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Neurological complications in aortic coarctation: Results of a Nationwide analysis based on 11,907 patients

Neurological complications in aortic coarctation: Results of a Nationwide analysis based on 11,907 patients View Article Trenk L, Lammers AE, Radke R, Baumgartner H, Wort SJ, Gatzoulis MA, Diller GP, Kempny A. Int J Cardiol. 2020 Aug 14:S0167-5273(20)33568-3. doi: 10.1016/j.ijcard.2020.08.041. Online ahead of print. PMID: 32798628 Take Home Points: In patients presenting for coarctation repair, the rate of spinal injury at the time of primary repair is low – 0.05-0.2% Patients with coarctation have a reduced survival rate compared to the general population Ongoing neurological complications, mainly in the form of subarachnoid bleeding and ischemic strokes occur in patients with repaired coarctation of the aorta Many patients had, at last follow-up, traditional risk factors for atherosclerosis Almost 25% had hypertension at last visit Subarachnoid bleeds occurred at a median age of 28.6 years Ischemic strokes displayed a bimodal peak with the median adult age 56.1 years The one-year mortality after a subarachnoid bleed was 16.2 % and post an ischemic infarct was 20%. Arterial hypertension (OR 3.75) was an independent risk factor for subarachnoid bleeding and both arterial hypertension (OR 4.10) and smoking (OR 13.46) emerged as independent risk factors for ischemic infarcts. This study findings emphasize the need for diligent ongoing care and control of risk factors for patients with repaired coarctation of the aorta Commentary from Dr. Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch:  Coarctation of the aorta represent 6-8% of congenital heart disease malformations. The timing and mode of clinical presentation depends largely on the severity of the lesion. Neurological complications following surgical or percutaneous have been reported. In this United Kingdom, nationwide retrospective study, the socio-demographic, clinical and surgical data of all patients being hospitalized with a diagnosis of coarctation of the aorta between 1997 and 2015 were reviewed. Data was obtained from the National Health Service Hospital Episode Statistics Database (HES, NHS Digital) using the appropriate search ICD-10 coding for the various diagnoses of interest. A total of 11 907 patients with coarctation of the aorta were identified. Almost 60% were male. During the period of 1997-2015, 8456 surgical or interventional procedures were performed. For the surgical procedure group, mortality on the same admission amounted to 2.5% (150 of 5905 surgeries) and the intervention mortality 0.3% (8 of 2550 interventions). The first operation was an end-to-end anastomosis in 56.9% of cases (n=2737), a subclavian flap in 12.5% (n=603) and a patch graft in 9.7%(n=469). Four hundred and seventy-two (9.8%) had percutaneous intervention as a first procedure. Neurological complications related to surgical or percutaneous intervention occurred in 10 patients (0.1% of all procedures): eight hemiplegia, one paralytic syndrome and one upper limb monoplegia. In those patients born after 1997, the neurological complication rate was 0.05% for surgeries and 0.21% for percutaneous interventions. For those with a revision, the complication rate was slightly higher – for a first revision, the surgical neurological complication rate was 0.24%. Cardiovascular risk factors in this cohort included: arteriosclerotic disease (3.3%), current smoking (5.3%), diabetes (1.7%), hyperlipidemia (3.5%), obesity (1.9%), renal dysfunction 4.3%), SVT’s (4.6%), and arterial hypertension in 24.5%.  Subarachnoid bleeds (n=37) and cerebral infarction (lnn= late neurological complications) were seen in 225 patients over a follow-up period of 146 295 patient-years at a rate of 0.15% per year. Subarachnoid bleeds occurred in 37 patients; the median age was 28.7 years (20.2-44.5 years). All but one had no history of a previously diagnosed cerebral aneurysm. 10.8% died on the same admission and the one year mortality in this complication was 16.2%. When matched for age and gender with those coarctation patients who did not experience a bleed, only arterial hypertension was an independent risk factor for bleeding (OR 3.75, 95% CI 1.25-11.3, p=0.019). Ischemic stroke occurred in 188 patients with a bimodal age distribution (peak in 1st year of life and then again over age 60). The median age of the adult population was 56.1 years. Index admission mortality was 6.9% and the one year mortality 20%. On multivariate analysis, arterial hypertension (OR 4.10, 95% CI 1.55-10.79, p=0.004) and smoking (OR 13.46 ,95% CI 1.57-115.35), p=0.018) remained adverse risk factors for ischemic stroke. The Kaplan -Meier curves show the greatest mortality rates are in the first year post-event but it continues to drop off in both cohorts.

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Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation

Risk Factors for Failed Fontan Procedure Following Stage 2 Palliation View Article Ono M, Burri M, Mayr B, Anderl L, Strbad M, Cleuziou J, Hager A, Hörer J, Lange R. Ann Thorac Surg. 2020 Aug 20:S0003-4975(20)31323-0. doi: 10.1016/j.athoracsur.2020.06.030. Online ahead of print. PMID: 32828751 Take Home Points: The outcome of Glenn shunt is generally good with the majority of the patients proceeding to Fontan completion. Hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure and a reduced ventricular function at the time of superior cavopulmonary shunt were identified as risk factors for failure to successfully complete the Fontan procedure. Atrio-ventricular valve regurgitation, despite associated with unbalanced atrioventricular septal defect and a reduced ventricular function, is not associated with failure to successful Fontan completion. Commentary from Dr. MC Leong (Kuala Lumpur, Malaysia), section editor of ACHD Journal Watch:  While the outcomes of total cavopulmonary connections are widely reported, there is a paucity of outcome data on the second stage single ventricular palliation. Such palliation includes fashioning of a superior cavopulmonary connection (bidirectional Glenn shunt) or in some centers, a hemi-Fontan procedure. This study aimed at analyzing the outcomes of all single ventricles which have undergone the second stage palliation and identifying the factors that are associated with death or failure to progress to a Fontan completion. This is a single center, retrospective review of a database of 525 patients with single ventricles who underwent bidirectional Glenn shunt at the German Heart Center in Munich between January 1998 and December 2018. Baseline characteristics of the patient population were as tabulated in Table 1.  The median time of Glenn shunt was 4.7 month (IQR, 3.0-7.4) while the median weight was 5.6 kg (IQR, 4.7-7.0). The perioperative data was summarized in Table 2. Of note is the patients with unbalanced atrioventricular septal defect were at greatest risk of early death (10.0%), whereas early death occurred in hypoplastic left heart syndrome patients was only 3.6% of all patients. Interestingly, in the early postoperative period, 55 patients required cardiac catheterization because of severe cyanosis and subsequent surgical/catheter interventions. The etiology for cyanosis were Glenn pathway stenosis (n=31), veno-venous collaterals (n=11), cavopulmonary pathway thrombus (n=4), and undetermined (n=9). In all 9 patients whose etiology was undetermined, placement of a systemic to the branch pulmonary shunt and performing a pulmonary septation to create unilateral Glenn shunt to the contralateral pulmonary artery was performed, out of which, five patients died postoperatively. Of the 514 early survivors, 15 patients were lost to follow-up after hospital discharge (Figure 1). The median follow-up period was 3.4 years (IQR, 1.5-8.7) for the remaining 499 patients. A competitive risk analysis was performed onto patients who had Fontan completion, death, and 'being alive without Fontan'. Patients who failed to achieve successful Fontan completion was defined as those who died before Fontan completion; patients who were considered unsuitable for Fontan completion; and patients who died early after Fontan completion (death within 30 days), because they failed to establish successful Fontan circulation. The cumulative incidence of Fontan completion (83.9% at 3 years and 87.1% at 5 years), the cumulative incidence of death (10.4% at 3 years and 10.7% at 5 years), and survival without Fontan completion (5.7% at 3 years and 2.2% at 5 years) are shown in Figure 2. Freedom from mortality before the Fontan procedure, unsuitability for the Fontan procedure, and early mortality after the Fontan procedure at 1, 2, and 3 years were 91.9%, 87.3%, and 86.1%, respectively. ] Figure 2. Multivariate analysis showed that hypoplastic left heart syndrome, unbalanced atrio-ventricular septal defect (AVSD), high pulmonary artery pressure and a reduced ventricular function at Glenn shunt were associated with failure to achieve an eventual successful Fontan completion. Although significant atrioventricular valve regurgitation requiring intervention was not identified as an independent risk in multivariate model, the incidence of atrioventricular valve intervention was significantly higher in patients with unbalanced AVSD (26.7% vs. 10.3%, p=0.006) and in patients with reduced ventricular function (26.1% vs. 9.8%, p=0.001). Apart from the conclusions that the study came to, this study also raised two important issues. (1) The timing of Glenn shunt needs to be balanced between overloading the ventricle with a systemic to pulmonary artery shunt and the need to grow the pulmonary artery to a caliber suitable for the eventual Fontan completion. In this study, the Glenn shunts were performed relatively early at the median age of 4.7 month (IQR, 3.0-7.4). Meanwhile, pulmonary artery reconstruction was required in 189 (36%) patients during Glenn shunt. As many as 129 (25%) patients required bilateral branch pulmonary arteries repair. It is possible that many of them required pulmonary artery intervention to enlarge the pulmonary arteries, which may alter the dimension of the pulmonary artery outside of the lungs but not the pulmonary arterial tree within the lungs. The calibers of the pulmonary arteries are not only anatomical but also physiologically linked to the long-term outcome of the eventual Fontan procedure; (2) If a patient presents with unexplained cyanosis post Glenn shunt, the prognosis is poor. In this study, 9 patients had no recognizable anatomic defect, and hypoxemia was perceived to be secondary to inadequate pulmonary blood flow. Implanting an additional systemic to pulmonary shunt and pulmonary artery septation which supposedly meant to improve oxygenation, unfortunately, yielded in 5 deaths. These patients may perhaps benefit from other treatment options.    

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Impact of preoperative electrophysiological intervention on occurrence of peri/postoperative supraventricular tachycardia following Fontan surgery

Impact of preoperative electrophysiological intervention on occurrence of peri/postoperative supraventricular tachycardia following Fontan surgery View Article Takeuchi D, Toyohara K, Kudo Y, Nishimura T, Shoda M. Heart Rhythm. 2020 Aug 8:S1547-5271(20)30756-6. doi:...

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Improvement in ventricular function with rhythm control of atrial arrhythmias may delay the need for atrioventricular valve surgery in adults with congenital heart disease.

Zielonka B, Kim YY, Supple GE, Partington SL, Ruckdeschel ES, Marchlinski FE, Frankel DS. Congenit Heart Dis. 2019 Aug 5. doi: 10.1111/chd.12833. [Epub ahead of print] PMID: 31385437 Similar articles Select item 31380593   Take Home Points: Atrial arrhythmias in ACHD...

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