Prenatal diagnosis of coarctation: Impact on early and late cardiovascular outcome.

BACKGROUND: Prenatal diagnosis (PND) of aortic coarctation (AoCo) has been associated with a significant improvement in early results, but there is limited information on the long-term cardiovascular outcome. METHODS: We studied 103 patients with simple AoCo, operated in the neonatal period, with a median follow-up of 8,5 years (2 to 23,7 years), with 47% followed for over 10 years. PND was made in 35%. The primary aim was to determine the short and long-term cardiovascular impact of PND of AoCo. RESULTS: Neonates with PND had less preoperative neonatal complications, with only 2,8% incidence of a composite preoperative severe morbidity course, compared to 28% in the postnatal group. PND patients underwent surgery 8 days earlier and had a shorter length of stay in ICU. PND did not impact the incidence of post-operative complications. On the long-term, prevalence of hypertension, left ventricular hypertrophy and rate of recoarctation were not influenced by PND. The PND group had mean 24 h diastolic BP 9 mmHg lower and mean daytime diastolic BP 11 mmHg lower. In the final multivariable model, PND was the single independent variable correlating with daytime diastolic BP. CONCLUSION: PND of AoCo effectively leads to a better pre-operative course with less pre-operative morbidity. We found no significant differences in immediate post-operative cardiovascular outcomes. A better initial course of patients with PND does not have a major long-term impact on cardiovascular outcomes, nevertheless, at late follow-up PND patients had lower diastolic BP values on ambulatory monitoring, which may have an impact on long-term cardiovascular risk.

Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: The nevada experience.

OBJECTIVE: We reviewed our center's experience with neonatal and infant hypoplastic aortic arch, unassociated with intracardiac malformations, and investigated changes in prenatal detection rates over time for those requiring therapeutic procedures. METHODS: We identified all prenatal diagnoses of hypoplastic aortic arch with situs solitus, unassociated with intracardiac malformations, made in Nevada between May 2017 and April 2022. In addition, we identified all those 0-180 days old, with prenatal care, that underwent a surgical or interventional cardiac catheterization aortic arch procedure, whether prenatally or postnatally diagnosed. We excluded those with ventricular septal defects, functionally univentricular hearts, interrupted aortic arches, or any associated malformation requiring an additional surgical or interventional procedure ≤6 months old. Additionally, we calculated prenatal detection rates for those undergoing a surgical or interventional catheterization procedure for each of the 5 years. RESULTS: We identified 107 patients prenatally and postnatally. Of the 107 patients, 56 (34 prenatally diagnosed and 22 postnatally diagnosed) underwent an aortic arch procedure, and 51 additionally prenatally diagnosed, live-born infants did not undergo a procedure. Of the 56 procedures, 2 were by interventional catheterization, and 54 underwent a surgical repair. Prenatal detection for those undergoing a procedure statistically significantly increased over the 5 years from 38% to 82%, rho = 0.95 (p = .04). CONCLUSIONS: Currently in Nevada, our prenatal detection rate is >80% in the general population for those between 0 and 6 months old who require a therapeutic procedure for aortic arch obstruction without intracardiac malformations.

Incidence and risk factors for late complications after the arterial switch operation.

OBJECTIVE: The purpose of this study was to evaluate our institution's 16-year arterial switch operation (ASO) experience and to determine early and late mortality and late morbidity, as well as the need for reoperation and catheter intervention, and finally, to explore risk factors for late complications and reintervention. METHODS: The clinical data of 185 transposition of the great arteries (TGA) patients who received ASO treatment in our center from January 2006 to January 2022 were continuously included for retrospective study. RESULTS: There were 13 early deaths (7.03%), 5 late deaths (3.01%), and 6 lost to follow-up. The median follow-up time for the 166 hospitalized survivors was 88.5 (2190) months. Moderate or above new aortic valve regurgitation (NAR; in this article, NAR represents moderate or greater reflux unless otherwise specified) occurred in 19 cases (11.45%), and aortic root dilation (ARD) occurred in 28 cases (16.87%). Late right ventricular outflow tract obstruction (RVOTO) occurred in 33 cases (19.88%). There were 18 patients (10.84%) who underwent late re-intervention, and the most common indication for intervention was RVOTO, followed by recurrent aortic coarctation in patients undergoing concurrent arch repair and NAR or ARD. Receiver operating characteristics analysis found that NAR had the strongest predictive power for ARD, followed by RVOTO, followed by bicuspid native pulmonary valve (BPV), and aorto-pulmonary diameter mismatch (APDMM) was the weakest. Multivariate analysis showed that APDMM, previous pulmonary artery banding (PAB), and mild NAR at discharge were independent risk factors for late NAR and ARD. Low surgical weight was an independent risk factor specific to NAR, and BPV was an independent risk factor specific to ARD. Older surgical age and ARD were independent risk factors for late RVOTO. Older surgical age, operation before 2014, late RVOTO, and late ARD were independent risk factors for late intervention. No reintervention events for coronary dysfunction were found in the late stage, but one patient occurred myocardial infarction due to coronary embolism after reoperation. CONCLUSION: Early and late survival rates after ASO in TGA patients have been remarkably improved in recent decades. Increased rates of NAR, ARD, recurrent coarctation of the aorta, and RVOTO as children age are major future outcomes of concern and may imply more late reinterventions. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with APDMM, previous PAB, mild NAR at discharge, low surgical weight, and BPV structures.

Implications of Left Ventricular Dysfunction at Presentation for Infants with Coarctation of the Aorta.

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted. Infants < 6 months old at diagnosis with aortic coarctation were identified using surgical codes for coarctation repair between January 2010 and May 2018. Moderate to severe dysfunction was defined as ejection fraction (EF) < 40%. Of 160 infants studied, 18 (11%) had moderate to severe LV dysfunction at presentation. Compared to those with better LV function, infants with moderate to severe LV dysfunction were older at presentation (12 vs. 6 days, p = 0.004), had more postoperative cardiac intensive care unit (ICU) days (5 vs. 3, p < 0.001), and more ventilator days (3.5 vs. 1, p < 0.001). The median time to normal LV EF (≥ 55%) was 6 days postoperatively (range 1-230 days). Infants presenting with moderate to severe LV dysfunction had higher index hospitalization costs ($90,560 vs. $59,968, p = 0.02), but no difference in cost of medical follow-up for the first year following discharge ($3,078 vs. $2,568, p = 0.46). In the current era, > 10% of infants with coarctation present with moderate to severe LV dysfunction that typically recovers. Those with moderate to severe dysfunction had longer duration of mechanical ventilation and postoperative cardiac ICU stays, likely driving higher costs of index hospitalization.

Analysis of factors associated with prolonged post-operative course after surgical repair of aortic coarctation.

OBJECTIVE: We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation. METHODS: We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay. RESULTS: We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay. CONCLUSIONS: In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.

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

BACKGROUND: Patients with coarctation of the aorta (CoA) are at increased risk of periprocedural and late neurological complications. Based on a nationwide inpatient dataset, we assessed the prevalence and risk factors for neurological complications in this cohort. METHOD AND RESULTS: We included all CoA patients hospitalized in England between 1997 and 2015. The risk for procedural complications, subarachnoid bleeding and ischemic stroke were quantified, and risk factors investigated. Overall, 11,907 patients (60% male) with CoA were identified. The median age at first presentation was 0.6 years with a median follow-up period of 14.4 years. Throughout the study period, 8456 surgical or interventional procedures were performed in 6796 patients. The neurological complication rate for primary surgical or interventional repair in patients born after 1997 was 0.05% and 0.2%, respectively. During a total follow-up period of 146,295 patient-years, late neurological complications such as subarachnoid bleeding and cerebral infarction occurred in 225 patients (0.15%/patient-year). The median age for subarachnoid bleeding was 29 years and 57 years for ischemic stroke. While, arterial hypertension (p = .0014), current smoking (p = .02), dyslipidaemia (p = .007) and diabetes (p = .037) were significantly related to the risk of ischemic stroke, only arterial hypertension emerged as significant risk factor for subarachnoid bleeding. CONCLUSIONS: Neurological complication during primary CoA repair are extremely rare in the current era. In contrast, many CoA patients are afflicted by late complications such as subarachnoid bleeding and ischemic stroke at a relatively young age, emphasizing the need for meticulous and pro-active blood pressure control and reduction of cardiovascular risk factors.

Epidemiology, Clinical Features, and Use of Early Supportive Measures in PHACE Syndrome: A European Multinational Observational Study.

BACKGROUND: PHACE syndrome is a rare inborn condition characterized by large facial hemangiomas and variable malformations of the arterial system, heart, central nervous system, and eyes. According to Orphanet estimates, the prevalence is <1.0 per million. Data from Europe are limited to small case series, and there are no population-based data available. OBJECTIVES: We conducted the present study to provide population-based estimates of the disease prevalence of PHACE syndrome in children in Germany, Switzerland, and Austria. We compared these first systematic data on PHACE syndrome from Europe to published data from the PHACE Syndrome International Clinical Registry and Genetic Repository (USA). Clinical features in our cohort with PHACE syndrome were assessed in detail, including the need for early supportive measures. METHODS: We used a population-based approach by means of a previously well-established network of child neurologists from Germany, Switzerland, and Austria ("ESNEK") to identify potential patients. The patients' guardians and child neurologists were asked to fill in questionnaires developed in collaboration with the International PHACE Registry. RESULTS: We identified 19 patients with PHACE syndrome. Estimated prevalence rates were 6.5 per million in Switzerland, 0.59 per million in Germany, and 0.65 per million in Austria. A subset of 10 patients from Germany and Switzerland participated in our study, providing detailed clinical assessment (median age: 2.5 years; 9 females, 1 male). Cerebrovascular involvement was frequent (80%). Facial hemangioma extent correlated significantly with the number of organs involved (p = 0.011). In 9 out of 10 patients, facial hemangiomas were treated successfully with oral propranolol. Baseline demographic data as well as the rate of cerebrovascular and cardiovascular anomalies were in line with those from the US International PHACE Registry and other published PHACE cohorts. CONCLUSIONS: Our study provides population-based estimates for PHACE syndrome in 3 German-speaking countries. The data from Switzerland indicate that PHACE syndrome may be more prevalent than demonstrated by previous reports. Underreporting of PHACE syndrome in Germany and Austria likely accounts for the differences in prevalence rates. The clinical observation of a potential association between the size of facial hemangioma and extent of organ involvement warrants further investigation.

Is Carotid Intima-Media Thickness Increased in Adults With Congenital Heart Disease?

Background Because of the increasing numbers of congenital patients surviving into adulthood, early diagnosis and prevention of acquired cardiovascular disease is reasonable. The aim of this study was to detect diagnostic subgroups of adults with congenital heart disease (ACHD) that have increased carotid intima-media thickness (cIMT), a subclinical marker of cardiovascular damage. Methods and Results This study enrolled 831 ACHD patients (392 women, aged 38.8±11.7 years) from May 2015 to February 2019 at their regular outpatient visit. Far wall cIMT was measured using a semiautomatic ultrasound system at 4 angles. Age, sex, height, weight, blood pressure, smoking status, and antihypertensive medication were registered and entered in a multiple linear regression model to compare diagnostic subgroups to 191 healthy controls (111 women, aged 36.7±13.5 years). There were no significant differences in cIMT of ACHD (0.538±0.086 mm) compared with healthy controls (0.541±0.083 mm; P=0.649) after adjusting for the aforementioned covariates. Only patients with coarctation of the aorta showed significantly higher cIMT values (0.592±0.075 mm; P<0.001) compared with healthy controls. In addition, ACHD patients who were men (P=0.032), older (P<0.001), and were prescribed antihypertensive medications (P=0.003) were all found to have thicker cIMT values. Conclusions Overall, we determined that within the ACHD cohort, only those patients with a history of coarctation have higher cIMT values. To better determine the mechanism of abnormal vasculature, further basic research is needed.

Increased risk of intraventricular hemorrhage in low birth weight infants with aortic coarctation.

Objective: To determine if there is an association between aortic coarctation (CoA) and intraventricular hemorrhage (IVH) in low birth weight (LBW) infants that may justify earlier intervention. While there is an increasing number of reports of successful early CoA intervention in LBW infants, there are no data to justify this approach.Material and methods: Retrospective review of the University Health System Consortium Clinical Data Base/Resource Manager, a national hospital discharge database. LBW (≤2 kg) infants, with and without IVH and isolated CoA were identified; IVH was stratified into low (grade 1 or 2) and high (grade 3 or 4) severity. Odds ratios were calculated for any, low and high-grade IVH with CoA.Results: Forty-six thousand and twenty LBW infants were identified; 3716 (8.1%) had IVH, 3001 (81%) with low, and 724 (19%) with high severity. Sixty-four infants had CoA, 13 had associated IVH (10 with low and three with high severity). The odds ratio for any IVH with CoA was 2.91 (95% CI 1.58-5.35), low severity 2.77 (95% CI 1.41-5.46) and high severity 3.45 (95% CI 1.07-11.07).Conclusions: In conclusion, this large retrospective database review found that LBW infants with CoA may be at increased risk of IVH. Further study is needed to determine if earlier catheter- or surgical-based intervention for CoA could reduce the risk of IVH in this population.

Surgical repair of aortic coarctation in adults: half a century of a single centre clinical experience.

OBJECTIVES: Coarctation of the aorta (CoA) is rarely diagnosed and treated in adults and nowadays mostly treated with percutaneous techniques. The objective of this study is to report the long-term outcomes and health-related quality of life in a unique cohort of patients treated with an open surgical repair for their primary adult CoA. METHODS: Ninety adult patients underwent primary surgical repair of CoA at our tertiary referral centre between 1961 and 2008 when the treatment strategy for adult CoA was exclusively surgical. RESULTS: The median age at surgery was 24 years (interquartile range 20-36 years), and 39 patients (43%) were asymptomatic at presentation. CoA was located paraductally in most patients (64%), and bicuspid aortic valve was present in 39 (43%) patients. Surgical reconstruction of CoA with an end-to-end anastomosis was performed in majority of the patients (57%). Overall, in-hospital mortality occurred in 1 patient (1%). There was no in-hospital stroke, spinal cord ischaemia, renal replacement therapy or respiratory failure. The cumulative survival was 97.7%, 89.5%, 82.6%, 70.9% and 61.4% at 10, 20, 30, 40 and 50 years, respectively. Thirty-one patients (34%) required an additional cardiac surgery during follow-up. The majority of patients (77%) suffered from refractory hypertension even after decades of surgery. Compared with the matched Dutch population, patients reported a lower social functioning, mental health, vitality and general health with a higher body pain. CONCLUSIONS: Patients with native adult CoA have low in-hospital morbidity and mortality when treated with an open surgical reconstruction. However, refractory hypertension and impaired quality of life remain important challenges during follow-up.

Procedural risk factors, incidence and timing of reintervention after treatment for native coarctation of the aorta in children: a population-based study†.

OBJECTIVES: The aim of the present study was to evaluate procedural risk factors, incidence and timing of reintervention because of recurrent aortic coarctation in children. METHODS: The study cohort consisted of 304 patients with isolated coarctation: 251 underwent surgery and 53 were treated percutaneously (40 balloon angioplasty, 13 stent) at the Helsinki Children's Hospital in 2000-2012. Characteristics, intervention and reintervention data were retrospectively collected from clinical records until 2014 (median follow-up 7.9 years). Age- and sex-matched comparisons between the treatment groups were performed in 86 patients (surgery n = 43, percutaneous n = 43). RESULTS: Forty of the 251 (16%) patients after surgery, 9/40 (23%) patients after balloon angioplasty and 4/13 (31%) patients after stent placement underwent a reintervention after a median time of 3.4, 11.7 and 19.5 months (P < 0.05), respectively. In the surgery group, all reinterventions occurred in children operated on ≤12 months of age and were related to lower body weight and smaller dimensions of the aorta. In the balloon angioplasty group, a higher post-procedure systolic arm-leg blood pressure gradient was associated with reintervention. After stent placement, three-fourths of the reinterventions were performed in a planned postinterventional catheterization. In the age- and sex-matched comparisons (median 5,7 years, range 0,5-17,6), post-procedure blood pressure gradients were higher (mean 10 vs 4 mmHg, P = 0.03), and reinterventions were more common (28%, 95% confidence interval 17-43 vs 2%, 95% confidence interval 0-12) in the percutaneous group compared to the surgery group. CONCLUSIONS: Reinterventions after surgery in neonates were relatively common. In older children, percutaneous treatment carried a higher risk of reinterventions, which were mainly related to residual coarctation after primary treatment.

Primary surgical repair of coarctation of the aorta in adolescents and adults: intermediate results and consequences of hypertension.

OBJECTIVES: Coarctation of the aorta is known to present with hypertension in older patients; we reviewed our experience and assessed the outcome of hypertension following surgical correction. METHODS: From April 2004 to date, 43 patients above the age of 12 underwent coarctation of the aorta repair. The mean age was 20.4 + 9.7 years (maximum 56 years); 21 (48.8%) were older than 18 years and 28 (65.1%) were men. Thirty (69.8%) patients had hypertension. Fourteen (32.6%) had a bicuspid aortic valve; 11 (25.6%) had patent ductus arteriosus; 6 (14%) had myxomatous mitral valve; 4 (9.3%) had ascending aortic aneurysms; and 2 (4.7%) had descending aneurysms. RESULTS: Surgical correction included resection and interposition of a tube graft in 31 (72.1%), an end-to-end anastomosis in 6 (14%) and patch aortoplasty in 3 (7%). Three (7%) patients required an extra-anatomical bypass: 1 had a long segment coarctation of the aorta, and 2 had a Bentall procedure with an ascending-to-descending aortic bypass. Staged procedures were done for concomitant disease in 4 (9.3%). There was 1 death: a 56-year-old woman died of refractory ventricular fibrillation during surgery. Thirty (69.8%) patients were discharged with antihypertensive medication. At a follow-up of 2.8 ± 2.2 years (maximum 9.2 years), the number of hypertensive patients decreased (17/36; 47.2%) (P = 0.042). Univariable predictors for persistence of hypertension revealed the use of an interpositional tube graft for repair (odds ratio 13.855, confidence interval 0.000-0.001; P = 0.001) as an indicator, whereas there were no independent predictors for persistence of hypertension. CONCLUSIONS: Surgical intervention is warranted irrespective of age and helps correct and control hypertension better; however, significant numbers of patients still require antihypertensive medication and regular monitoring. Intervention using an interposition tube graft may affect the prevalence of hypertension.

Subclinical Burden of Coronary Artery Calcium in Patients With Coarctation of the Aorta.

Coronary computed tomography (CT) angiography is often performed in adults with coarctation of the aorta (CoA) for anatomic assessment. As this population ages, assessment of atherosclerotic cardiovascular disease burden is important. Thus, quantitative and qualitative coronary artery calcium (CAC) scores were assessed for patients with CoA ≥16 years of age, who were seen at a referral center. CoA patients had either coronary CT angiography or chest CT with interpretable coronary information performed for clinical indications (follow-up, preoperative, or for symptoms) from 2004 to 2017. Qualitative CAC was determined based on low-dose CT and lung cancer screening protocols. Quantitative CAC scores were compared with an age- and gender-matched control cohort of patients chosen from an emergency department database of patients who received coronary CT angiography for chest pain evaluation. Atherosclerotic cardiovascular disease 10-year predicted risk scores were calculated for both cohorts. Out of 131 patients with CoA (mean age 46.1 ± 15.3 years), 22 patients (17%) had multivessel atherosclerotic disease on qualitative assessment. In the subgroup of patients ≥40 years, those with CoA were more likely to have a quantitative CAC score ≥400 compared with those without CoA (14% vs 4%, p = 0.02). Median atherosclerotic cardiovascular disease risk score was 8% (interquartile range 2% to 12%) for CoA patients ≥40 years, and 5% (interquartile range 2% to 9%) for patient without CoA ≥40 years. In conclusion, we determined that CoA patients have subclinical atherosclerosis identifiable on CT in high rates when compared with patients without CoA. Atherosclerotic cardiovascular disease should be assessed in these patients for prevention and treatment.

Prenatal Risk Factors for PHACE Syndrome: A Study Using the PHACE Syndrome International Clinical Registry and Genetic Repository.

The cause of PHACE syndrome is unknown. In a study of 218 patients, we examined potential prenatal risk factors for PHACE syndrome. Rates of pre-eclampsia and placenta previa in affected individuals were significantly greater than in the general population. No significant risk factor differences were detected between male and female subjects.

Infantile Aortic Coarctation in an Adult with Heart Failure.

OBJECTIVE: The aim of this case report is to highlight the importance of excluding aortic coarctation in a patient with a bicuspid aortic valve. CLINICAL PRESENTATION AND INTERVENTION: A 56-year-old woman with hypertension was admitted complaining of progressive dyspnea. Transthoracic echocardiography showed a calcified, bicuspid aortic valve with severe stenosis. Aortography revealed the presence of severe narrowing of the aorta between the carotid and subclavian arteries. The patient was referred for cardiovascular surgery in which successful aortic valve replacement and aortic correction were performed. CONCLUSION: This case report shows an uncommon finding of infantile aortic coarctation in an adult patient admitted with heart failure.

Recurrent Coarctation After Neonatal Univentricular and Biventricular Norwood-Type Arch Reconstruction.

BACKGROUND: Previous studies have evaluated recurrent coarctation after the Norwood procedure (NP) in patients with single-ventricle (SV) anatomy. Extended patch augmentation of the aorta, a Norwood-type arch reconstruction (NTAR), is also used in patients with 2 ventricles and a diffusely hypoplastic arch; however, outcomes after NTAR in these patients are not well described. METHODS: A retrospective review was performed of neonates with SV and 2-ventricle (2V) anatomy who underwent an NP or NTAR at our institution from 2000 to 2010. The incidence of recurrent coarctation requiring intervention and associated risk factors were evaluated. RESULTS: Overall, 101 patients with 2V anatomy and 361 patients with SV anatomy were included. Eighteen patients with 2V anatomy (17.8%) and 35 patients with SV anatomy (9.7%) required intervention for recurrent coarctation at a median of 0.5 years (interquartile range [IQR], 0.3-1.2 years) after the initial operation. Independent risk factors associated with recurrent coarctation in patients with 2V anatomy included weight less than 2.5 kg (hazard ratio [HR], 6.05; p = 0.001) and peak aortic arch gradient (PAAG) on the discharge echocardiogram greater than 10 mm Hg (HR, 3.07; p = 0.03). In patients with SV anatomy, shunt type (HR, 6.42; p < 0.0001 for right ventricle to pulmonary artery [RV-PA] shunt compared with others) and peak gradient on the discharge echocardiogram greater than 10 mm Hg were found to be significant (HR, 7.40; p < 0.0001). There was no survival difference in patients with and those without recurrent coarctation. CONCLUSIONS: Recurrent coarctation is common after NTAR. Small patient size and shunt type were found to be independent risk factors for recurrent coarctation in the 2V and SV populations, respectively, and discharge aortic arch gradient was a significant risk factor in both populations.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS: There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS: The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.

Prevalence and Clinical Characteristics of Headaches in PHACE Syndrome.

PHACE (posterior fossa brain malformation, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities) syndrome is a neurocutaneous disorder often involving the cerebral vasculature. PHACE patients appear to have early-onset and severe headaches more commonly than children without PHACE. The objective of this study was to characterize the clinical features and prevalence of headache by conducting a cross-sectional survey of families in 2 large PHACE registries. Sixty-six percent of eligible families completed the survey in which 62.7% of respondents reported headaches. Average age of headache onset was 48.8 months. Females were more likely to have headaches (68.6% vs 30.8%, P = .014). Families reported associated migrainous features including nausea (62.5%), vomiting (37.5%), photophobia (75%), and phonophobia (75%). Headaches occurred at least weekly in 29.4%, lasted ≥1 hour in 85.4%, and led to ≥1 hospital admission in 15.7%. Three respondents with headaches had at least 1 ischemic stroke. We demonstrated that headaches are common among PHACE patients, develop at an early age, and have migrainous features.

Office-Based Subglottic Evaluation in Children With Risk of Subglottic Hemangioma.

PURPOSE: Children with V3 cutaneous infantile hemangiomas (IH) and PHACE syndrome have a high incidence for airway hemangioma, 29% and 52%, respectively. Therefore, a clinical evaluation for these high-risk children is essential. We report our experience with in-office lower airway evaluation (OLAE) in these high-risk children. RESULTS: Since 2003, 5 children with IH of the V3 cutaneous distribution and 3 children with PHACE syndrome underwent OLAE. Average age of presentation was 2.75 months. Two children had stridor at initial evaluation, and 1 child had subglottic hemangioma. This child was evaluated serially with OLAE to monitor disease progression and treatment response. A total of 10 upper tracheoscopies were performed on the 8 patients without respiratory complications. CONCLUSION: An airway evaluation is essential to evaluate and manage this high-risk population. Typically, operative endoscopy requires general anesthesia. However, in these high-risk children, we have performed OLAE without sedation to evaluate the trachea. High-speed recording and playback is essential in this method. Our series demonstrates that awake OLAE is possible and may be a safe technique to evaluate and monitor disease progression in these high-risk patients. These patients avoided general anesthesia and delay in diagnosis and did not incur any complications during or after OLAE.

Long-term Follow-up of "Simple" Lesions--Atrial Septal Defect, Ventricular Septal Defect, and Coarctation of the Aorta.

Surgery for congenital heart disease has advanced significantly in the past 50 years, such that repair of "simple" lesions, such as atrial septal defect, ventricular septal defect, and coarctation of the aorta carries minimal risk, with mortality risk much less than 1%. It was once thought successful repair of these lesions was definitively corrective. There is mounting evidence, however, that there are long-term complications after these repairs, prompting the need for continued follow-up. This review describes the current understanding of diagnosis, treatment, and long-term outcomes for these patients, with the goal of advocating for lifelong surveillance. As the perioperative care of these repairs has evolved significantly over time, so must the way in which we study these patients in the long-term.