Antipsychotic prescribing: national findings of children and adolescents attending mental health services in Ireland.

Antipsychotic prescribing trends vary internationally, albeit off-label use remains high (i.e., target symptoms). We aim to describe antipsychotic use, target conditions, target symptoms and dosing regimens in children and adolescents in Ireland. We used a sampled cohort from a national audit of children and adolescents attending mental health services with predefined inclusion and exclusion criteria from Jul-2021 to Dec-2021 who were prescribed at least one psychotropic medication and up to and including 17-years of age (n = 3528). Each service provided anonymised data. We described the frequency of antipsychotic medication, medication type, target condition, target symptom and medication doses. We used multivariable logistic regression, adjusted with available co-variates to assess the association of being prescribed an antipsychotic medication. Twelve percentage (n = 437) were prescribed an antipsychotic and 16-17-years (n = 211, 48.3%) was the most common age category. The commonest reason for prescribing an antipsychotic was target symptoms (i.e., off-label use) (n = 329; 75.%) and of these symptoms, agitation (n = 77/329; 25%) and irritability (56/239; 25%) were the most common. Quetiapine (n = 127; 29%) was the most common antipsychotic, followed by risperidone (n = 125; 28.6%), aripiprazole (n = 107; 24.5%), and olanzapine (n = 66; 15.1%). In adjusted analysis, having a psychotic disorder ((adjusted-odds-ratio) aOR: 39.63, CI 95%, 13.40-117.22), bipolar disorder (aOR: 16.96, CI 95%, 3.60-80.00), autism spectrum disorder (aOR: 3.24, CI 95%, 2.45-4.28) or aggression symptoms (aOR: 16.75, CI 95%, 7.22-38.89) was associated with prescribing an antipsychotic medication. This is the first study in children and adolescents that describes the target conditions and target symptoms for antipsychotic use in Ireland. Our results show a high proportion of antipsychotic prescribing based on target symptoms rather than target condition or diagnosis.

Household energy poverty and trajectories of emotional and behavioural difficulties in children and adolescents: findings from two prospective cohort studies.

PURPOSE: The aim of this study is to examine the association between household energy poverty (EP) and trajectories of emotional and behavioural difficulties during childhood. METHODS: The Growing up in Ireland study is two nationally representative prospective cohorts of children. The Infant Cohort (n = 11,134) were recruited at age 9 months (9 m) and followed up at 3, 5, 7 and 9 years (y). The Child Cohort (n = 8,538) were recruited at age 9 y and followed up at 13 y and 17/18 y. EP was a composite of two relative measures of EP. Emotional and behavioural difficulties were repeatedly measured using the strengths and difficulties questionnaire (SDQ). Linear spline multilevel models were used, adjusted for confounders to examine the association between (1) EP (9 m or 3 y) and trajectories of emotional and behavioural difficulties from 3 to 9 y in the Infant Cohort and (2) EP at 9 y and the same trajectories from 9 to 18 y in the Child Cohort. RESULTS: In adjusted analyses, EP at 9 m or 3 y of age was associated with higher total difficulties score at 3 y (0.66, 95% CI 0.41, 0.91) and 5 y (0.77, 95% CI 0.48, 1.05) but not at 7 y or 9 y. EP at 9 y was associated with higher total difficulties score at 9 y (1.73, 95% CI 1.28, 2.18), with this difference reducing over time leading to 0.68 (95% CI 0.19, 1.17) at 17/18 y. CONCLUSIONS: Our study demonstrates a potential association between early life EP and emotional and behavioural difficulties that may be transient and attenuate over time during childhood. Further studies are required to replicate these findings and to better understand if these associations are causal.

National Clinical Programme for Eating Disorders: a pragmatic review of a new national eating disorder service in Ireland.

OBJECTIVE: The Health Service Executive National Clinical Programme for Eating Disorders (NCPED) launched a Model of Care for Eating Disorder Services in Ireland in 2018. Currently, one adult and two child and adolescent eating disorder services are operational out of a total of sixteen recommended. The three objectives of this paper are to describe the early (1) referral pattern, (2) level of service activity and (3) the level of service user satisfaction. METHOD: Monthly submitted service activity data from each service to the NCPED between March 2018 and October 2020 were retrospectively analysed. One hundred and fifty-nine carers and service users completed an experience of service questionnaire (ESQ). A descriptive analysis of referral pattern, level of service activity and ESQ was performed. A thematic analysis was performed on three qualitative questions on the ESQ. RESULTS: There was substantial referral numbers to eating disorder services by 18 months (n = 258). The main referral source was community mental health teams. The majority (n = 222, 86%) of referrals were offered an assessment. The most common age profile was 10-17 years of age (n = 120, 54.1%), and anorexia nervosa was the most common disorder (n = 96, 43.2%). ESQ results demonstrate that most service users were satisfied with their service, and the main themes were carer involvement, staff expertise, therapeutic alliance and service access. CONCLUSIONS: This preliminary service activity and service user satisfaction data highlight several issues, including trends when setting up a regional eating disorder service, potential pitfalls of pragmatic data collection and the need for adequate information-technology infrastructure.

Acute Kidney Injury and Renal Replacement Therapy After Fontan Operation.

Fontan circulation leads to chronic elevation of central venous pressure. We sought to identify the incidence, risk factors, and survival among patients who developed acute kidney injury (AKI) after the Fontan operation. We retrospectively reviewed 1,166 patients who had Fontan operation/revision at Mayo Clinic Rochester from 1973 to 2017 and identified patients who had AKI (defined by AKI Network criteria) within 7 days of surgery. A total of 132 patients (11%) developed AKI after the Fontan operation with no significant era effect. Of those who developed AKI, severe (grade 3) kidney injury was present in 101 patients (76.5%). Multivariable risk factors for AKI were asplenia (odds ratio [OR] 4.2, p <0.0001), elevated preoperative pulmonary artery pressure (per 1 mm Hg increase, OR 1.04, p = 0.0002), intraoperative arrhythmias (OR 1.9, p = 0.02), and elevated post-bypass Fontan pressure (per 1 mm Hg increase, OR 1.12, p = 0.0007). Renal replacement therapy (RRT) was used in 72 patients (54%), predominantly through peritoneal dialysis (n = 56, 78%). Multivariable risk factors for RRT were age ≤3 years (OR 9.7, p = 0.0004), female gender (OR 2.6, p = 0.02), and aortic cross-clamp time >60 minutes (OR 3.1, p = 0.01). Patients with AKI had more postoperative complications, including bleeding, stroke, pericardial tamponade, low cardiac output state and cardiac arrest, than those without AKI. This resulted in longer intensive care unit stay (39 vs 17 days, p = 0.0001). In-hospital mortality was exceedingly higher among patients with AKI versus no AKI (58%, 76 of 132 vs 10%, 99 of 1,034, p <0.0001); however, there was no significant difference based on the need for RRT. Recovery from AKI was observed in 56 patients (42%). Over 20-year follow-up, patients with AKI had a distinctly higher all-cause-mortality (82%) than those without AKI (35%). It is prudent to identity patients at a higher risk of developing postoperative AKI after Fontan operation to ensure renal protective strategies in the perioperative period. Postoperative AKI leads to substantial short and long-term morbidity and mortality, but the need for RRT does not affect the outcomes.

Venous thromboembolic and bleeding complications among pregnant women with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a vascular malformation overgrowth syndrome characterized by capillary malformation, venous malformation, and limb overgrowth, with or without lymphatic malformation. Patients are at an increased risk of hemorrhage and venous thromboembolism (VTE). Consequently, women with this condition often are counseled to avoid pregnancy, but minimal data are available on the relationship between pregnancy, VTE, and bleeding risk. OBJECTIVE: To review the risk of VTE and bleeding in pregnant and nulligravid women with KTS. METHODS: A retrospective medical record review was performed of women with KTS, aged ≥18 years, evaluated at Mayo Clinic Rochester, Minnesota, from August 1945 to April 2018. RESULTS: We identified 75 women with ≥1 pregnancy and 64 nulligravid women. VTE prevalence was 14 of 70 (20%) for women with a history of pregnancy and 16 of 64 (25%) for nulligravid women (P = .93). Among the 70 women with a history of pregnancy, 7 of 18 VTE events (39%) occurred in association with pregnancy, with VTE affecting 7 of 151 pregnancies (4.6%). Significant bleeding prevalence was 6 of 70 (8.6%) for women with a history of pregnancy and 6 of 64 (9.4%) for nulligravid women (P = .54). LIMITATIONS: This was a retrospective review. CONCLUSION: The prevalence of VTE and bleeding was similar in patients with KTS, irrespective of pregnancy status.

Total Knee Arthroplasty Is Safe and Successful in Patients With Klippel-Trénaunay Syndrome.

BACKGROUND: Klippel-Trénaunay syndrome (KTS) is a severe vascular malformation that can lead to hypertrophic osteoarthritis. Total knee arthroplasty (TKA) performed in extremities affected with KTS is challenging given the high-risk vascular considerations and occasionally poor bone quality. METHODS: We identified 12 patients with KTS who underwent TKA between 1998 and 2017. There were 7 men, mean age 42 years, and mean follow-up was 7 years. Before arthroplasty, 2 patients (17%) had preoperative sclerotherapy. Preoperative vascular studies were done for 9 patients (75%) and included magnetic resonance imaging (n = 7), magnetic resonance angiography (n = 1), and computed tomography angiography (n = 1). A preoperative blood conservation protocol was used for all operations and included the use of tranexamic acid (TXA) in later years. Posterior-stabilized TKA was used in 10 cases and cruciate-retaining TKA was used in 2 cases. RESULTS: At final follow-up, 2 patients (17%) had undergone revision surgery: 1 for infection and 1 for tibial loosening with subsequent arthrofibrosis. Knee Society Scores (36-83, P < .0001) and functional scores (48-84, P = .0007) significantly increased between the preoperative and postoperative period. Likewise at last follow-up, the mean knee range of motion significantly increased (82°-104°, P = .04). Median blood loss for patients who received TXA was 200 mL compared to 275 mL in patients who did not receive TXA (P = .66). Likewise there was no difference (P = .5) in the proportion of patients who required a transfusion between those who received TXA (2/6, 33%) and those who did not (3/6, 50%). CONCLUSION: In this small series, TKA can lead to significant clinical improvement for patients with KTS. Modern blood management techniques and a careful multidisciplinary care approach render TKA a reasonable option for select patients with KTS. LEVEL OF EVIDENCE: Level IV case series, therapeutic.

Fontan Failure Secondary to Charcot-Marie-Tooth-Induced Phrenic Neuropathy.

Charcot-Marie-Tooth disease comprises a vast array of defects in myelin integrity that causes progressive peripheral sensorimotor neuropathy. It is the most prevalent inherited peripheral neuropathy, and it can affect the management of coexisting medical conditions. We report the case of a 25-year-old woman who had undergone successful Fontan surgery during childhood, but her Fontan circulation failed as a result of diaphragmatic paresis caused by Charcot-Marie-Tooth disease type 1A. This diagnosis precluded cardiac transplantation.

Pain, psychiatric comorbidities, and psychosocial stressors associated with Klippel-Trenaunay syndrome.

BACKGROUND: Klippel-Trenaunay syndrome (KTS) is characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. Patients with KTS have lower scores in general in mental health, physical function, and quality of life than the general population. OBJECTIVE: To determine the prevalence of pain and psychiatric comorbidity in patients with KTS. METHODS: A retrospective review of 410 patients with KTS evaluated during 1976-2012 was conducted to identify the presence of pain, psychiatric comorbidities, and psychosocial stressors. RESULTS: Pain was reported by 260 patients (63.4%) and was associated with any KTS complication (P < .0001) and venous malformations of the lower extremities (P = .0008) and feet (P = .0007). Ninety-five patients had a diagnosed psychiatric condition (23.2%), most commonly depression (15.1%) and anxiety (5.1%). Pain (P = .0016), superficial thrombosis (P = .0269), deep embolic/thrombotic events (P = .0005), gastrointestinal complications (P = .0085), genitourinary complications (P = .0163), and capillary malformation of the hands (P = .0040) were associated with having a psychiatric diagnosis. LIMITATIONS: This is a retrospective study that relied on physician detection and reporting of variables. CONCLUSION: Pain and psychiatric conditions, particularly depression and anxiety, are common in patients with KTS. Awareness of the psychosocial impact of KTS and appropriate screening are important.

Idiopathic Restrictive Cardiomyopathy in Children and Young Adults.

Idiopathic restrictive cardiomyopathy (IRC) is a rare condition characterized by reduced ventricular compliance. Children with IRC have poor outcomes with most patients proceeding to cardiac transplantation. We sought to analyze our institutional experience and assess contemporary outcomes for children with IRC. We reviewed the medical record for patients (<21 years old) evaluated for a primary diagnosis of IRC between 1975 and 2013 at our institution. Demographic, clinical, echocardiographic, and catheterization data were abstracted. The patients were divided into 2 groups comprising a historical cohort (HC) (diagnosis: 1975 to 1993, n = 8) and a contemporary cohort (CC) (diagnosis: 1994 to 2013, n = 12). Twenty children were identified with IRC (mean age at presentation 9.7 ± 6.5 years, 55% female). Mean length of follow-up was 6.5 ± 8.4 years (range 0.1 to 35.6 years). In the CC, 7 of 12 patients (58%) progressed to cardiac transplantation (mean age of 9 ± 4 years at transplant, mean interval from diagnosis of IRC: 1.5 ± 0.9 years). Overall survival was improved significantly in the CC compared with the HC (80% vs. 38%, p = 0.02), but transplantation free survival was no different between the CC and HC over 5 years (38% vs 38%, p = 0.65). In the CC, elevation of mitral valve Doppler E/e' ratio on echocardiography was associated with increased mortality (p = 0.01). In conclusion, IRC continues has a poor prognosis. Early referral for transplantation was associated with improved overall survival in the modern era. Patients with markedly elevated E/e' ratio may have increased risk of death.

Mild prenatal hypoxia-ischemia leads to social deficits and central and peripheral inflammation in exposed offspring.

Hypoxic-ischemic encephalopathy (HIE) resulting from intrauterine or perinatal hypoxic-ischemia (HI) is a leading cause of long-term neonatal neurodisability. While most studies of long-term outcome have focused on moderate and severe HIE in term infants, recent work has shown that those with mild HIE may have subtle neurological impairments. However, the impact of mild HI on pre-term infants is much less clear given that pre-term birth is itself a risk factor for neurodisability. Here we show that mild HI insult alters behaviour, inflammation and the corticosterone stress response in a rat model of pre-term HIE. Mild HI exposure led to social deficits in exposed offspring at postnatal day 30, without impairments in the novel object recognition test nor in the open field test. This was also accompanied by elevations in circulating adrenocorticotropic hormone and corticosterone indicating an exaggerated stress response. There were also elevations in il-1ß and il-6 but not tnf-α mRNA and protein in the brain and blood samples. In summary we find that a mild HI exposure leads to social deficits, central and peripheral inflammation, and an abnormal corticosterone response which are three core features of autism spectrum disorder. This shows that mild HI exposure may be a risk factor for an abnormal neurodevelopmental outcome in pre-term offspring.

Management of refractory chylous ascites with peritoneovenous shunts.

OBJECTIVE: The purpose of this study was to define outcome of treatments of refractory chylous ascites using peritoneovenous shunts (PVSs). METHODS: Clinical data of patients with refractory chylous ascites treated with PVSs between 1992 and 2015 were retrospectively reviewed. The primary end point was clinical benefit, defined as cured, improved, or poor results; secondary end points were complications and reinterventions. RESULTS: Seventeen patients (eight female [47%]; median age, 47 years [range, 19-78 years]) with refractory chylous ascites were studied. This group represented 6% of 284 patients treated for chylous ascites during the study period. The etiology was primary lymphangiectasia in 10 patients (59%) and secondary chylous ascites due to previous surgery, lymphatic obstruction with associated portal hypertension, or malignant tumor in 7 (41%). Eleven patients were treated with LeVeen shunts and six with Denver shunts. Thirty-day mortality, morbidity, and reintervention rates were 5.9%, 18%, and 12%, respectively. Reintervention rate at 6 months was 9.1% with LeVeen shunt, significantly lower than 100% with Denver shunt (P = .001). During a mean follow-up of 5.1 years (range, 17 days-22.7 years), 7 of 11 patients with LeVeen shunt and all 6 patients with Denver shunt required shunt replacement. Median duration of patency was 215 days (range, 2 days-9.0 years) of a total of 25 LeVeen shunts placed in 11 patients and 44 days (range, 6-91 days) of 20 Denver shunts placed in 6 patients. At last follow-up, patency of the LeVeen shunt was 36% (4/11); symptoms improved in 64% of the patients (7/11). Patency rate of Denver shunts was 33% (2/6), and symptoms improved in 33% (2/6). CONCLUSIONS: Treatment of refractory chylous ascites continues to be a major challenge. The only currently available PVS, the Denver shunt, had a median patency period of <2 months; it required frequent replacements and resulted in intermittent short-term clinical benefit in one-third of the patients. Improvements in technology to design new shunts, to develop new therapies, or to adopt new techniques to treat chylous ascites are urgently needed.

Cardiopulmonary exercise test in adults with prior Fontan operation: The prognostic value of serial testing.

BACKGROUND: The purpose of the study was to determine the role of cardiopulmonary exercise test (CPET) indices in predicting cardiovascular adverse events (CAEs) in patients with Fontan palliation. CAE was defined as death or cardiac surgery. METHODS: Retrospective review of adult Fontan patients who had treadmill CPET at Mayo Clinic, 1994-2013. Patients with loss of follow-up defined as ≥2years without clinical follow-up were excluded. The results of serial CPETs were reviewed, and patients with CPETs meeting the following criteria were selected for analysis: maximum effort on serial CPETs, minimum of 3-year interval between CPETs, and absence of CAE between CPETs. RESULTS: A total of 145 patients met inclusion criteria for the study; age at baseline CPET was 24±3years; age at Fontan operation was 11±5years; and 91 (63%) were males. Baseline peak oxygen consumption (VO2) was 22.7±5.4ml/kg/min (63±11% predicted), peak heart rate was 135±31beats per minute, and oxygen saturation at peak exercise was 86±7%. Serial CPETs were performed in 71/145 patients (49%); mean duration between CPETs was 3.8±0.3years. The % predicted peak VO2 decreased by 1.7±0.9 percentage points/year. CAE (deaths n=22; cardiac surgery n=45) occurred in 54/145 patients (37%) within 8±3years. Decline in % predicted peak VO2≥3 percentage points/year was the only predictor of 5-year risk of CAE (HR 1.86, 95% CI 1.11-3.48, P=0.02). CONCLUSIONS: Serial CPET is prognostic of CAE in the adult Fontan population, and can be used to risk stratify these patients.

Surveillance for liver complications after the Fontan procedure.

The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population.

Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation.

There are limited studies of thrombotic and embolic complications (TEC) in the adult Fontan population. The purpose of the study was to determine the prevalence, risk factors, and outcomes of TECs in this population. METHODS: Retrospective review of adults with a previous Fontan operation, with follow-up at Mayo Clinic, 1994-2014. Systemic TEC was defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus. Nonsystemic TEC was defined as Fontan conduit/right atrial thrombus or pulmonary embolus. RESULTS: We identified 387 patients with a mean (SD) age of 28 (7) years and a mean follow-up of 8 (2) years. An atriopulmonary connection (APC) was done for 286 patients (74%). Atrial arrhythmias were present in 278 (72%). There were 121 TECs (systemic n=36, nonsystemic n=85) in 98 patients (25%). Risk factors for systemic TEC were atrial arrhythmia (hazard ratio 2.28, P=.001) and APC (hazard ratio 1.98, P=.02); nonsystemic TEC also had similar risk factors. All 98 patients received warfarin. Warfarin was discontinued in 10 of 98 because of bleeding, and 8 of these 10 subsequently had a second TEC. Among the 82 patients who had follow-up imaging, 16 (20%) had resolution of thrombus. In total, 24 of 98 patients had a second TEC, most of whom had inadequate anticoagulation. CONCLUSIONS: Thrombotic and embolic complication was not uncommon; risk factors for TEC were APC and atrial arrhythmias. Most patients were treated successfully with warfarin alone. A second TEC occurred in most patients whose anticoagulation was discontinued because of bleeding events.

Sudden cardiac death and late arrhythmias after the Fontan operation.

OBJECTIVES: We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation. BACKGROUND: Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation. METHODS: We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n = 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment >30 days after operation. RESULTS: We included 996/1052 (95%) patients with no arrhythmia diagnosis prior to Fontan. Overall 10-, 20-, and 30-year freedom from arrhythmias was 71%, 42%, and 24%, respectively. Of 864 patients who survived >30 days after Fontan, 304 (35%) had atrial flutter, 161 (19%) had atrial fibrillation, 108 (13%) had atrial tachycardia, 37 (4%) had reentrant supraventricular tachycardia, 40 (5%) had ventricular tachycardia, and 113 (13%) had sinus node dysfunction. Predictors of late arrhythmias included an atriopulmonary Fontan, age at operation (>16 years) or atrial arrhythmias postoperatively. During follow-up, 52/1052 (5%) patients had SCD, with 51 having documentation available; 8 patients died suddenly within 30 days and the remaining 43 had an average time to SCD of 6.9 ± 6.7 years (median was 3.8 years). Arrhythmias were documented in 28/43 (65%) patients prior to SCD. Predictors of SCD included atrioventricular valve replacement and post-bypass Fontan pressures >20 mm Hg; preoperative sinus rhythm was protective. CONCLUSIONS: Arrhythmias and SCD are significant concerns among Fontan patients and specific risk factors may warrant closer follow-up and earlier consideration for therapy.

Thrombotic and Embolic Complications Associated With Atrial Arrhythmia After Fontan Operation: Role of Prophylactic Therapy.

BACKGROUND: There are limited data about the risk of thrombotic and embolic complication (TEC) in adults with atrial arrhythmia after Fontan operation. OBJECTIVES: This study sought to determine the risk of TEC in this population and the role of anticoagulation therapy in TEC prevention. METHODS: This was a retrospective review of adults with atrial arrhythmia after Fontan operation who were evaluated at the Mayo Clinic between 1994 to 2014. TEC was classified into 2 groups: systemic TEC, defined as intracardiac thrombus, ischemic stroke, or systemic arterial embolus; and nonsystemic TEC, defined as Fontan conduit/right atrial thrombus or pulmonary embolus. Patients were divided into 3 groups: anticoagulation, antiplatelet, and no therapy cohorts. RESULTS: We followed 278 patients, mean age 31 ± 9 years, for 88 ± 14 months (1,464 patient-years). Patient groups included antiplatelet (n = 181), anticoagulation (n = 91), and no therapy (n = 6). There were 97 TEC in 81 patients (29%); 32 were systemic, yielding an event rate of 2.1 systemic TEC per 100 patient-years, and 65 were nonsystemic TEC, yielding an event rate of 4.4 nonsystemic TEC per 100 patient-years. Prevalence of TEC was 18% and 55% at 5 and 10 years, respectively. Atriopulmonary connection was a risk factor for TEC (hazard ratio: 2.31; 95% confidence interval: 1.61 to 4.64), and TEC were associated with higher risk of death and hospitalization (p < 0.0001). Anticoagulation was protective against TEC and resulted in a reduction of TEC risk by 2.5 TEC per 100 patient-years. Anticoagulation was also associated with lower risk of death and hospitalization (p = 0.02). Bleeding complications occurred in 21 (7%) patients and were similar in all groups. CONCLUSIONS: Anticoagulation was associated with lower TEC rate and lower risk of death and hospitalization, without a significant increase in bleeding risk. Perhaps anticoagulation should be the preferred preventive strategy.

Heart transplantation after Fontan: Results from a surgical Fontan cohort.

We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart-lung, one heart-liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post-bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non-transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post-transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate-term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.

Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia. CDH survivors may have pulmonary morbidity that can decrease cardiopulmonary exercise. We aimed to examine whether cardiopulmonary exercise testing (CPET) results differ in CDH survivors versus healthy age-matched controls and whether CPET results among CDH survivors differ according to self-reported daily activity. METHODS: In one medical center in Croatia, CDH survivors-patients with surgically corrected CDH who were alive at age 5 years-were invited to participate in spirometry and CPET. Values were compared with those of controls matched 2:1 by age and sex for each CDH survivor aged 7 years or older. RESULTS: Among 27 CDH survivors aged 5-20 years, 13 (48%) had continued symptoms or spirometric evidence of pulmonary disease. Compared with controls (n = 44), survivors (n = 22) had lower peak oxygen consumption (V˙O2 mean [SD], 35.7 [6.9] vs. 45.3 [8.2] ml/kg per min; P < 0.001). At peak exercise, V˙O2/heart rate (P < 0.001), tidal volume (P = 0.005), and minute ventilation (P < 0.001) were lower in survivors, but the maximal respiratory rate was not different (P = 0.72). Among survivors, mean (SD) V˙O2peak (ml/kg per min) differed by self-reported activity level: athletic, 40.3 (5.0); normal, 35.8 (6.5); and sedentary, 32.1 (6.8) (by ANOVA, P = 0.10 across three groups and P = 0.04 athletic vs. sedentary). CONCLUSION: More than half of CDH survivors continue to have chronic pulmonary disease. CDH survivors had lower aerobic exercise capacity than controls. Self-reporting information on daily activities may identify CDH patients with low V˙O2max who may benefit from physical training. Pediatr Pulmonol. 2016;51:1320-1329. © 2016 Wiley Periodicals, Inc.

Contraception Practices and Pregnancy Outcome in Patients after Fontan Operation.

OBJECTIVE: The feasibility and safety of pregnancy after the Fontan operation is not well understood. We sought to determine contraception practices and early and late outcomes of pregnancy after the Fontan operation. DESIGN: We performed a retrospective review of medical records to identify women of childbearing age from the Mayo Clinic Fontan database. A follow-up questionnaire was mailed to all patients not known to be deceased at the time of study. Patients with available contraception and pregnancy data were included in the study. RESULTS: Of the 138 women with available contraception data, 44% used no contraception, 12% each used barrier methods, combination hormone therapy or sterilization, 8% used Depo-Provera, 7% had intrauterine devices, 4% had a partner with a vasectomy and 1% used progestin pills. Six women had thrombotic complications (only one using oral contraceptives). Thirty-five women had pregnancy data available. Prior to the Fontan operation there were 10 pregnancies (8 miscarriages, 2 therapeutic abortions, and no live births). After the Fontan operation there were 70 pregnancies resulting in 35 miscarriages (50%), 29 live births (41%), and 6 therapeutic abortions (9%). There were no maternal deaths during pregnancy. During long-term follow up (26 ± 6 years since the Fontan), 1 death, and 1 cardiac transplant occurred. Mean gestational age of the newborns (n = 22/29) was 33.1 ± 4.0 weeks; mean birth weight (n = 20/29) was 2086 ± 770 g. There was 1 neonatal death because of prematurity and two children were born with congenital heart disease (one patent ductus arteriosus and one membranous ventricular septal defect). CONCLUSIONS: Pregnancy after the Fontan operation is associated with a high rate of miscarriages, preterm delivery, and low birth weight. Further studies are needed to identify specific variables influencing risk stratification of pregnancy in this patient population.