Poor concordance between definitions of type III arch and implications for risk prediction and assessment for carotid artery stenting.

OBJECTIVE: The type III arch configuration has been inconsistently reported as a stroke risk factor during carotid artery stenting. However, at least three different methods for the definition of type III arch can be identified in the literature, related to the level of the origin of the innominate artery (IA). According to Casserly's definition, a type III arch presents with an origin of the IA below the horizontal plane of the inner curvature. According to Madhwal's definition, a type III arch has a distance greater than twice the diameter of the left common carotid artery between the highest point of the arch and the origin of the IA. According to MacDonald's definition, a type III arch presents with a distance of ≥2 cm between the highest point of the arch and the origin of the IA. Our aim was to assess the level of concordance between these different methods. METHODS: Anonymized thoracic computed tomography scans of 100 healthy patients were reviewed. Two of us independently stratified the selected cases as a type I to III arch, according to the three considered definitions. The interobserver level of concordance for each type III arch classification and level of concordance among the three definitions were assessed. RESULTS: The 100 selected patients (64% male) were 76 ± 7 years old. For each definition, the interobserver repeatability was almost perfect for all three (Madhwal, κ = 0.81; 95% confidence interval [CI], 0.71-0.99; MacDonald, κ = 0.82; 95% CI, 0.72-0.92; Casserly, κ = 0.84; 95% CI, 0.74-0.93). The level of concordance among the different definitions was very low (Madhwal vs MacDonald, 85% [P = .002]; 33% for type III arch; Madhwal vs Casserly, 60% [P < .0001]; 12% for type III arch; MacDonald vs Casserly, 75% [P < .0001]; 12% for type III arch). CONCLUSIONS: The three definitions of the type III arch have a very low level of concordance, which might account for the varying clinical relevance of this configuration. Our findings have relevant implications for risk prediction for carotid artery stenting based on the presence of a type III arch, for comparisons of the results from different studies, and for comparisons of different datasets from multicenter trials.

Assessment of CT angiographic findings in comparison with echocardiography findings of chest among patients with aortic arch anomalies.

The objective of this study was to examine the findings of computed tomographic (CT) angiography in patients with aortic arch anomalies in comparison with transthoracic echocardiography findings who referred to a private imaging center in Tehran during 2009-2012. The cases included 203 patients with clinical symptoms or echocardiogram of congenital heart disease to assess the presence of aortic arch anomalies among patients referred to imaging center. This study is a retrospective study based on the CT angiographic findings in comparison with transthoracic echocardiography findings of chest among patients with aortic arch anomalies. In this study, 203 patients with congenital anomalies were enrolled in the study, among those, 107 patients were men and 96 were female. The most common anomaly of the aortic arch was found to be coarctation (19.7%), followed by right sided arch with mirror image branching (19.2%). Furthermore, the most common cardiac anomalies associated with aortic arch anomalies were VSD, PA and PDA. The sensitivity and specificity of transthoracic echocardiography in the diagnosis of aortic arch anomalies was 59% and 100% in comparison with CT angiography. In addition, the agreement between the two methods (kappa) in the diagnosis of aortic arch anomalies was 0.72. But, transthoracic echocardiography is the first diagnostic method for patients with congenital heart disease. In some patients, the ability of this method was limited to the detection of coronary artery anomalies and thoracic vessels. Therefore, CT is used for morphological evaluation of congenital heart disease (CHD) due to its main advantages including fast acquisition time, large anatomical coverage, high speed, and great spatial resolution. Moreover, CT is essential for proper evaluation of CHD regarding its high spatial and temporal resolution.

Assessment of intracardiac and extracardiac anomalies associated with coarctation of aorta and interrupted aortic arch using dual-source computed tomography.

To evaluate the value of dual-source computed tomography (DSCT) compared with transthoracic echocardiography (TTE) in assessing intracardiac and extracardiac anomalies in patients with coarctation of aorta (CoA) and interrupted aortic arch (IAA). Seventy-five patients (63 with CoA and 12 with IAA) who received preoperative DSCT and TTE were retrospectively studied. Intracardiac and extracardiac anomalies were recorded and compared by DSCT and TTE, in reference to surgical or cardiac catheterization findings. A total of 155 associated anomalies were finally found. Collateral circulation (56, 74.70%), patent ductus arteriosus (PDA; 41, 54.67%) were the most common anomalies. PDA, aortopulmonary window, and collateral circulation were more frequently present in patients with IAA than those with CoA (100% vs. 46.03%, 16.67% vs. 0%, and 100% vs. 69.84%, respectively, all p < 0.05). DSCT was superior to TTE in assessing associated extracardiac-vascular anomalies (sensitivity: 100% vs. 39.81%; specificity: 100% vs. 100%; positive predictive value: 100% vs. 100%; negative predictive value: 100% vs. 76.06%). Extracardiac-vascular anomalies, including collateral circulation and PDA, were the most common anomalies in patients with IAA and CoA. Compared with TTE, DSCT is more reliable in providing an overall preoperative evaluation of morphological features and extracardiac anomalies for surgical planning.

Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study.

OBJECTIVES: Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch. METHODS: Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 [age <18 years, n = 51] or the Short Form [SF]-36 [age ≥18 years, n = 66]) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis. RESULTS: Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution. CONCLUSIONS: Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.

Prenatal echocardiographic assessment of right aortic arch.

OBJECTIVES: To report our experience with fetal diagnosis of right aortic arch (RAA) variants based on the ductus arteriosus (DA) anatomy and brachiocephalic vessel branching pattern in relation to the trachea, and to establish whether the echocardiographic 'V-shaped' or 'U-shaped' appearance of the junction between the DA and aortic arch (AA) in the fetal upper mediastinal view is sufficiently accurate for assessment of fetal AA anatomy. METHODS: This was a retrospective study of pregnancies with a prenatal diagnosis of fetal RAA that had postnatal confirmation of AA anatomy, referred to our tertiary center during 2011-2017. Prenatal and postnatal medical records, including echocardiographic and computed tomography (CT)/magnetic resonance imaging (MRI) scan reports, were reviewed, and cardiac and extracardiac abnormalities and the results of genetic testing were recorded. RESULTS: Of 55 consecutive pregnancies with a prenatal diagnosis of fetal RAA, six were lost to follow-up, one was terminated and three were excluded due to lack of postnatal confirmation of AA anatomy. Of the remaining 45 pregnancies, AA anatomy was assessed postnatally by CT in 39, by MRI in one and by direct examination at cardiac surgery in five. A U-shaped appearance was found in 37/45 (82.2%) patients, all of which had a complete vascular ring (CVR). Of these 37 patients, on postnatal confirmation, 21 (56.8%) had RAA with Kommerell's diverticulum, left posterior ductus arteriosus (LPDA) and aberrant left subclavian artery (ALSA) (RAA/LPDA/ALSA), 11 (29.7%) had a double AA (DAA), four (10.8%) had RAA with Kommerell's diverticulum, LPDA and mirror-image (MI) branching (RAA/LPDA/MI), and one (2.7%) had RAA with Kommerell's diverticulum, LPDA and aberrant left innominate artery (ALIA) (RAA/LPDA/ALIA). A V-shaped appearance was found in 3/45 (6.7%) patients, all of which had RAA with right DA not forming a CVR and MI branching. In the 5/45 (11.1%) fetuses with neither U- nor V-shaped appearance, RAA with left anterior DA arising from the left innominate artery and MI branching, not forming a CVR, was found. Twelve (26.7%) fetuses had a congenital heart defect (CHD). RAA forming a CVR (U-shaped appearance) was associated with a septal defect in 6/37 (16.2%) fetuses, while RAA not forming a CVR (V-shaped appearance or no U- or V-shaped appearance) was associated with major CHD in 6/8 (75.0%) fetuses. CONCLUSIONS: In fetuses with RAA, V-shaped appearance of the junction between the DA and AA indicates only that the transverse AA and DA run together on the same side of the thorax (trachea) while a U-shaped appearance is always a sign of a CVR. Among fetuses with a CVR, RAA/LPDA/MI is more frequent than described previously. Finally, RAA forming a CVR is not usually associated with complex CHD, as opposed to RAA not forming a CVR. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Catheter-based anatomic and functional assessment of coronary arteries in anomalous aortic origin of a coronary artery, myocardial bridges and Kawasaki disease.

Most diagnostic testing in patients with anomalous aortic origins of coronary arteries, myocardial bridges, and coronary artery changes after Kawasaki disease are performed with the use of noninvasive techniques. In some cases, however, further diagnostic information is needed to guide the clinician in treating these patients. In such instances, cardiac catheterization with invasive anatomic and functional testing is an invaluable tool. Moreover, interventional treatment in the cardiac catheterization laboratory may be performed in a small subset of these patients. As the diagnosis of these conditions is now becoming more common, it is important for pediatric interventional cardiologists to be familiar with these techniques. In this article, the role of angiography, intravascular ultrasound, fractional flow reserve, and optical coherence tomography in these patients is reviewed.

Assessment of children with vascular ring.

BACKGROUND: Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic methods and treatment results after surgery in children with vascular ring. METHODS: Symptomatic vascular ring patients undergoing surgery between January 2010 and August 2014 at Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, were retrospectively assessed. The presentation symptoms, demographic characteristics, and diagnostic tests were evaluated. Operative data and postoperative follow up, complications and problems were also examined in detail. RESULTS: Twenty-one patients underwent surgery, 13 (63%) of whom were male. Median age was 12 months (range, 1 month-8 years). Among these patients, 62% (n = 13) had double aortic arch, 24% (n = 5) had right-sided aortic arch + ligamentum arteriosum, 10% (n = 2) had left-sided aortic arch + aberrant right subclavian artery, and 4% (n = 1) had pulmonary sling abnormality. Computed tomography (CT) angiography was performed in 17/21 patients. All the patients were successfully operated on. In the early postoperative period, two patients had chylothorax and one had nerve paralysis of nervus laryngeus recurrens. One patient died in the early period, and two patients had ongoing stridor on follow up. CONCLUSIONS: CT angiography is effective for the differential diagnosis and visualization of vascular ring abnormality. In such cases, full recovery can be assured with early diagnosis and surgery.

Useful signs for the assessment of vascular rings on cross-sectional imaging.

Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the compressed airway and esophagus are not encircled by patent, opacified vessels, there are useful secondary signs that should be considered and should raise suspicion for the presence of a vascular ring. These signs include a double aortic arch, the four-vessel sign, the distorted subclavian artery sign, a diverticulum of Kommerell, a ductal diverticulum contralateral to the aortic arch, and a descending aorta contralateral to the arch or circumflex aorta. If none of these findings is present, a ring can be excluded with confidence.

Evolución clínica de pacientes pediátricos post-tratamiento de coartación aórtica / Clinical evolution of pediatric patients post-treatment of aortic coarctation

La Coartación Aórtica representa del 5% - 10% de las malformaciones congénitas cardiovasculares. Lamayoría se localizan, en la pared posterior de la aorta (yuxtaductales) Objetivo: Establecer evolución clínicapost tratamiento de coartación aórtica, en el Hospital General de Enfermedad del Instituto Guatemalteco deSeguridad Social. Metodología: Estudio descriptivo, retrospectivo, en pacientes <15 años de edad, condiagnóstico de coartación aórtica que ya hubieran recibido tratamiento. Resultados: Se encontraron 39pacientes. 21 de sexo masculino y 18 de sexo femenino; 18 pacientes (9 masculino / 9 femenino) fuerontratados por intervencionismo; 12 pacientes con tratamiento medico. Las complicaciones fueron 10pacientes con recoartación y 7 pacientes con hipertensión arterial, el 100% en clase funcional NYHA tipo I y0% de mortalidad. Conclusiones: Los pacientes post tratamiento de coartación de aorta, cursaron conevolución clínica adecuada, 43% de ellos tuvo algún tipo de complicación, la mortalidad fue 0%.(

Aortic Coarctation represent 5 % - 10 % of congenital cardiovascular malformations. Most are located in theposterior wall of the aorta (yuxtaductal) Objective: To establish clinical outcome after treatment of aorticcoarctation in the General Hospital of Disease in Guatemalan Institute of Social Security. Methodology: Descriptive, retrospective study, in patients <15 years of age, diagnosed with aortic coarctation who hadalready received treatment. Results: 39 patients were found. 21 male and 18 female, 18 patients (9 male / 9female) were treated for interventional cardiology, 12 patients with medical treatment. Complications werepresent in 10 patients with Re coarctation and 7 patients with hypertension, 100% in NYHA functional class Itype and 0% mortality. Conclusions: The post treatment of aortic coarctation patients, presented with goodclinical course, 43 % of them had some type complication, and mortality was 0 %.

Two- and three-dimensional transthoracic echocardiography in the assessment of aortic arch vasum vasi to pulmonary artery fistula.

We report an elderly patient presenting with a stroke and also hemolytic anemia secondary to mitral and tricuspid valve repair 3 years earlier, in whom two-dimensional transesophageal echocardiography (2DTEE) suspected a fistula into the pulmonary artery (APAF) from the posterior wall of the aortic arch. For further assessment, two-dimensional transthoracic echocardiography (2DTTE) and live/real time three-dimensional transthoracic echocardiography (3DTTE) were performed. 2DTTE showed findings similar to 2DTEE. Color Doppler guided continuous-wave Doppler showed continuous flow throughout the cardiac cycle with systolic preponderance across the fistula with a small peak pressure gradient of 22 mmHg. Sequential systematic cropping of the 3D datasets obtained from 3DTTE confirmed that the flow signals originated from the posterior wall of the aortic arch and not the lumen. Thus, 3DTTE served to increase the confidence level that the fistula most likely originated from a vasum vasi in the wall of the aortic arch. To our knowledge, this type of fistula has not been reported previously. Fistulas from the aortic lumen to the pulmonary artery are also rare and so far only 24 cases have been reported in the literature. These are also reviewed by us.

Two-year neurodevelopmental outcomes of infants undergoing neonatal cardiac surgery for interrupted aortic arch: a descriptive analysis.

OBJECTIVE: This study determined neurodevelopmental outcomes of survivors of neonatal cardiac surgery for interrupted aortic arch through an interprovincial program and explored preoperative, intraoperative, and postoperative outcome predictors. METHODS: Children who underwent neonatal cardiac surgery for interrupted aortic arch at 6 weeks old or younger between 1996 and 2006 had a multidisciplinary neurodevelopmental assessment at 18 to 24 months old (mental and psychomotor developmental indices as mean +/- SD and delay [score <70]). Survivor outcomes were compared by univariate and multivariate analyses and compared between children with and without chromosomal abnormality. RESULTS: Outcomes were available for all 26 survivors (mortality, 3.7%). Mental and psychomotor developmental indices were 75.8 +/- 17.1 and 72.3 +/- 16.9, respectively, with significantly lower scores for children with chromosomal abnormalities, which accounted for 29% of the variance in developmental indices. For the remaining 17 children without chromosomal abnormalities, mental and psychomotor developmental indices were 82.7 +/- 14.5 and 79.1 +/- 14.3, respectively, with deep hypothermic circulatory arrest time and Apgar score at 5 minutes contributing 46% of the variance in mental developmental index. CONCLUSIONS: The neurodevelopmental indices of children who have undergone neonatal cardiac surgery for interrupted aortic arch are below normative values; those of children with chromosomal abnormalities are even lower. For children without a chromosomal abnormality, longer deep hypothermic circulatory arrest times and low Apgar scores predict lower mental developmental indices at 18 to 24 months of age.

MDCT assessment of tracheomalacia in symptomatic infants with mediastinal aortic vascular anomalies: preliminary technical experience.

BACKGROUND: Mediastinal aortic vascular anomalies are relatively common causes of extrinsic central airway narrowing in infants with respiratory symptoms. Surgical correction of mediastinal aortic vascular anomalies alone might not adequately treat airway symptoms if extrinsic narrowing is accompanied by intrinsic tracheomalacia (TM), a condition that escapes detection on routine end-inspiratory imaging. Paired inspiratory-expiratory multidetector CT (MDCT) has the potential to facilitate early diagnosis and timely management of TM in symptomatic infants with mediastinal aortic vascular anomalies. OBJECTIVE: To assess the technical feasibility of paired inspiratory-expiratory MDCT for evaluating TM among symptomatic infants with mediastinal aortic vascular anomalies. MATERIALS AND METHODS: The study group consisted of five consecutive symptomatic infants (four male, one female; mean age 4.1 months, age range 2 weeks to 6 months) with mediastinal aortic vascular anomalies who were referred for paired inspiratory-expiratory MDCT during a 22-month period. CT angiography was concurrently performed during the end-inspiration phase of the study. Two pediatric radiologists in consensus reviewed all CT images in a randomized and blinded fashion. The end-inspiration and end-expiration CT images were reviewed for the presence and severity of tracheal narrowing. TM was defined as > or =50% reduction in tracheal cross-sectional luminal area between end-inspiration and end-expiration. The presence of TM was compared to the bronchoscopy results when available (n = 4). RESULTS: Paired inspiratory-expiratory MDCT was technically successful in all five patients. Mediastinal aortic vascular anomalies included a right aortic arch with an aberrant left subclavian artery (n = 2), innominate artery compression (n = 2), and a left aortic arch with an aberrant right subclavian artery (n = 1). Three (60%) of the five patients demonstrated focal TM at the level of mediastinal aortic vascular anomalies. The CT results were concordant with the results of bronchoscopy in all patients who underwent bronchoscopy (n = 4). CONCLUSION: Paired inspiratory-expiratory MDCT is technically feasible for evaluating TM in symptomatic infants with mediastinal aortic vascular anomalies and has the potential to facilitate prompt diagnosis and treatment.

The role of magnetic resonance imaging in the assessment of suspected extrinsic tracheobronchial compression due to vascular anomalies.

AIMS: To evaluate the role of magnetic resonance imaging (MRI) in the assessment of children with suspected extrinsic tracheobronchial compression due to vascular anomalies. METHODS: Retrospective case note review in a tertiary referral centre. Twenty nine children who underwent dynamic laryngotracheobronchoscopy (DLTB) and were found to have a clinical suspicion of extrinsic tracheobronchial compression were evaluated. All subsequently underwent thoracic MRI within 10 days. The findings on endoscopy were compared to those of MRI, and where performed, echocardiography, aortography, and surgery. RESULTS: There were 17 males and 12 females (mean age 5 months, range 28 weeks gestation to 60 months). The most common presenting features were stridor and cyanotic episodes. MRI showed abnormalities in 21 patients. There were five vascular rings (three double aortic arches and two right aortic arches) and 11 cases of innominate artery compression. Other vascular anomalies noted included aberrant right subclavian artery and aneurysmal left pulmonary artery. Echocardiography was generally found to be unhelpful in the diagnosis of extra-cardiac vascular abnormalities. Angiography was subsequently conducted in eight children; findings agreed with those shown on MRI. Surgery was performed on all five vascular rings, one innominate artery compression, and one aneurysmal left pulmonary artery. Surgical findings were also compatible with the preoperative MRI. CONCLUSIONS: This study shows the successful use of MRI as the initial imaging modality in endoscopically suspected extrinsic vascular compression of the upper airway. It enables accurate delineation of vascular anomalies and, unlike aortography, is non-invasive and does not require the use of contrast media.

Intracardiac echocardiographic guidance for hemodynamic assessment in a patient with congenital abnormalities and a prosthetic aortic valve.

In this case report, we present the use of intracardiac echocardiography (ICE) for guiding the cardiac catheterization and subsequent hemodynamic investigation in an unusual patient case with multiple congenital abnormalities (bicuspid aortic valve, left cervical aortic arch, two aortic coarctations) and two aortic valve replacement operations in the past. The ICE catheter (AcuNav) permitted us to accurately and safely puncture the interatrial septum and place the Swan-Ganz catheter in the left ventricle; additionally, visualization of the aortic coarctation in the ascending aorta was also achieved.

Echocardiographic assessment of interrupted aortic arch.

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.

A genetic assessment of trisomy 21 in a patient with persistent truncus arteriosus who died 38 years ago.

A newborn baby with the unique combination of Down syndrome and persistent truncus arteriosus with interrupted aortic arch is described. The diagnosis of trisomy 21 was assessed 38 years after the patient's death by means of fluorescent in situ hybridization.