Pulmonary Atresia with Intact Ventricular Septum: Correlation of Preoperative Computed Tomography-Derived Parameters with Echocardiographic Tricuspid Valve Z-Score and Surgical Outcomes.

The role of preoperative cardiac computed tomography (CT) in neonates with pulmonary atresia and intact ventricular septum (PA-IVS) remains unclear. This study was aimed to elaborate the role of preoperative CT-derived anatomical and functional findings in planning treatment strategies in neonates with PA-IVS. The presence of ventriculocoronary arterial connections was evaluated by CT. CT-derived ventricular volumetric parameters were compared and correlated with echocardiographic tricuspid valve (TV) z-score in 12 neonates with PA-IVS. Cardiac CT and echocardiographic findings were compared between definite surgical types (median follow-up, 4 years). Ventriculocoronary arterial connections were identified with CT in 58.3% of cases (7/12) and associated with higher incidence of Fontan procedure (42.9%, 3/7) and high mortality (28.6%, 2/7). The CT-derived and echocardiographic TV z-scores exhibited a high correlation (R = 0.924, p < 0.001). The CT-derived right ventricle (RV) volume and RV-left ventricle volume ratio also displayed high correlations (R = 0.875 and 0.867, respectively; p < 0.001) with echocardiographic TV z-score. More positive echocardiographic TV z-score, high CT-derived RV end-diastolic volume and RV-left ventricle volume ratio, and low CT-derived left ventricular end-diastolic volume were observed in biventricular surgery group (N = 2), compared to Fontan operation (N = 3) and 1.5 ventricular surgery (N = 3) groups, and mortality cases (N = 3). Preoperative CT-derived coronary artery anatomy and ventricular volumetric parameters may supplement treatment planning in neonates with PA-IVS especially when multifactorial decision including echocardiographic TV z-score is in a gray zone.W.

Contemporary multimodality non-invasive cardiac imaging protocols for tetralogy of Fallot.

Tetralogy of Fallot is the most prevalent cyanotic congenital heart disease, requiring lifelong multimodality non-invasive cardiac imaging, such as echocardiography, cardiothoracic computed tomography, and cardiac magnetic resonance imaging. As imaging techniques continuously evolve and are gradually integrated into clinical practice, there is a critical need to update multimodality imaging protocols. Over the last two decades, cardiothoracic computed tomography imaging techniques have advanced remarkably, significantly enhancing its role in evaluating patients with tetralogy of Fallot. In this review, we describe contemporary multimodality non-invasive cardiac imaging protocols for tetralogy of Fallot, emphasizing the expanding role of cardiothoracic computed tomography. Additionally, we present standardized reporting forms designed to facilitate the clinical adoption of these protocols.

Pediatric three-dimensional quantitative cardiovascular computed tomography.

High-resolution, isotropic, 3-dimensional (D) data from pediatric cardiovascular computed tomography (CT) offer great potential for the accurate quantitative evaluation of pediatric cardiovascular and pulmonary vascular diseases. Recent pilot studies using pediatric 3-D cardiovascular CT have shown promising results in assessing cardiac function in conditions such as tetralogy of Fallot, cardiac defects with a hypoplastic ventricle, Ebstein anomaly, and in quantifying myocardial mass. In addition, the quantitative assessment of pulmonary vascularity is useful for evaluating differential right-to-left pulmonary vascular volume ratio, the effectiveness of pulmonary angioplasty, and predicting pulmonary hypertension. These initial experiences could broaden the role of pediatric cardiovascular CT in clinical practice. Furthermore, the current barriers to its widespread use, pertinent solutions to these problems, and new applications are discussed. In this review, the 3-D quantitative evaluations of cardiac function and pulmonary vascularity using high-resolution pediatric cardiovascular CT data are illustrated.

Fast Quantitative Magnetic Resonance Imaging Evaluation of Hydrocephalus Using 3-Dimensional Fluid-Attenuated Inversion Recovery: Initial Experience.

OBJECTIVE: This study aimed to demonstrate the initial experience of using fast quantitative magnetic resonance imaging (MRI) to evaluate hydrocephalus. METHODS: A total of 109 brain MRI volumetry examinations (acquisition time, 7 minutes 30 seconds) were performed in 72 patients with hydrocephalus. From the measured ventricular system and brain volumes, ventricle-brain volume percentage was calculated to standardize hydrocephalus severity (processing time, <5 minutes). The obtained values were categorized into no, mild, and severe based on the fronto-occipital horn ratio (FOHR) and the ventricle-brain volume percentages reported in the literature. The measured volumes and percentages were compared between patients with mild hydrocephalus and those with severe hydrocephalus. The diagnostic performance of brain hydrocephalus MRI volumetry was evaluated using receiver operating characteristic curve analysis. RESULTS: Ventricular volumes and ventricle-brain volume percentages were significantly higher in in patients with severe hydrocephalus than in those with mild hydrocephalus (FOHR-based severity: 352.6 ± 165.6 cm 3 vs 149.1 ± 78.5 cm 3 , P < 0.001, and 26.8% [20.8%-33.1%] vs 12.1% ± 6.0%, P < 0.001; percentage-based severity: 359.5 ± 143.3 cm 3 vs 137.0 ± 62.9 cm 3 , P < 0.001, and 26.8% [21.8%-33.1%] vs 11.3% ± 4.2%, P < 0.001, respectively), whereas brain volumes were significantly lower in patients with severe hydrocephalus than in those with mild hydrocephalus (FOHR-based severity: 878.1 ± 363.5 cm 3 vs 1130.1 cm 3 [912.1-1244.2 cm 3 ], P = 0.006; percentage-based severity: 896.2 ± 324.6 cm 3 vs 1142.3 cm 3 [944.2-1246.6 cm 3 ], P = 0.005, respectively). The ventricle-brain volume percentage was a good diagnostic parameter for evaluating the degree of hydrocephalus (area under the curve, 0.855; 95% confidence interval, 0.719-0.990; P < 0.001). CONCLUSIONS: Brain MRI volumetry can be used to evaluate hydrocephalus severity and may provide guide interpretation because of its rapid acquisition and postprocessing times.

Complimentary Cardiac Computed Tomography Ventricular Volumetry-Derived Metrics of Severity in Patients with Ebstein Anomaly: Comparison with Echocardiography-Based Severity Indices.

Detailed three-dimensional cardiac segmentations using cardiac computed tomography (CT) data is technically feasible in patients with Ebstein anomaly, but its complementary role has not been evaluated. This single-center, retrospective study was aimed to evaluate the complementary role of cardiac CT ventricular volumetry in evaluating the severity of Ebstein anomaly. Preoperative cardiac CT ventricular volumetry was performed in 21 children with Ebstein anomaly. CT-based ventricular functional measures were compared between Carpentier types, and between definitive surgical repair types. The Celermajer severity index measured with echocardiography was correlated with CT-based functional parameters. Total right ventricle (RV) and functional RV (fRV) volumes, fRV fraction, fRV/left ventricle (LV) volume ratio, and end-diastolic CT severity index demonstrated statistically significant differences between Carpentier type A/B and Carpentier type C/D (p < 0.05). The Celermajer severity index measured with echocardiography showed a high positive correlation with the end-diastolic CT severity index (R = 0.720, p < 0.002). There were no statistically significant differences in both echocardiography- and CT-based functional measures between patients with biventricular repair and patients with one-and-a-half or univentricular repair (p > 0.05). Compared with echocardiography, cardiac CT ventricular volumetry can provide the severity of Ebstein anomaly objectively and may be used in select patients when echocardiographic results are inconclusive or inconsistent.

Pediatric Cardiovascular Computed Tomography: Clinical Indications, Technique, and Standardized Reporting. Recommendations From the Cardiothoracic Taskforce of the European Society of Pediatric Radiology.

Congenital heart diseases affect 1% of all live births in the general population. The prognosis of these children is increasingly improving due to advances in medical care and surgical treatment. Imaging is also evolving rapidly to assess accurately complex cardiac anomalies prenatally and postnatally. Transthoracic echocardiography is the gold-standard imaging technique to diagnose and follow-up children with congenital heart disease. Cardiac computed tomography imaging plays a key role in the diagnosis of children with congenital heart defects that require intervention, due to its high temporal and spatial resolution, with low radiation doses. It is challenging for radiologists, not primarily specialized in this field, to perform and interpret these studies due to the difficult anatomy, physiology, and postsurgical changes. Technical challenges consist of necessary electrocardiogram gating and contrast bolus timing to obtain an optimal examination. This article aims to define indications for pediatric cardiac computed tomography, to explain how to perform and report these studies, and to discuss future applications of this technique.

Partial voxel interpolation to reduce partial volume error of cardiac computed tomography ventricular volumetry in patients with congenital heart disease.

BACKGROUND: Varying degrees of partial volume error depending on the complexity of the endocardial borders are inevitable in threshold-based cardiac computed tomography (CT) ventricular volumetry. These errors can potentially be reduced by using a partial voxel interpolation (PVI) method, but this has not been tested for cardiac CT ventricular volumetry. OBJECTIVE: To evaluate the partial volume error-reducing effects of the PVI method in cardiac CT ventricular volumetry among patients with congenital heart disease (CHD). MATERIALS AND METHODS: The cardiac CT ventricular volumetry data were obtained from 55 patients (median age 12.0 years) with CHD. The ventricular and myocardial volumes, ejection fraction and ventricular mass-volume ratio were quantified and compared before and after the PVI method. The correlation between the myocardial volumes in the end-systolic and end-diastolic phases was tested. The effect of the PVI method on the classification of ventricular hypertrophy was evaluated. RESULTS: The indexed ventricular volumes after PVI were significantly smaller (7.4-11.5%) than those before PVI (P<0.001). In contrast, the indexed myocardial volumes were significantly larger (6.2-27.7%) after PVI (P<0.001). The ejection fractions and mass-volume ratios were significantly larger (1.6-2.2% and 19.7-42.5%, respectively) after PVI (P<0.001 and P<0.001, respectively). The indexed myocardial masses showed prominently high correlation between the end-systolic and end-diastolic phases (R, 0.961-0.990; P<0.001). The proportions of no and severe hypertrophy were significantly decreased (P<0.002) and increased (P<0.032), respectively, after the application of the PVI method. CONCLUSION: The PVI method can reduce partial volume error in cardiac CT ventricular volumetry among patients with CHD.

Multimodality diagnostic imaging for anomalous pulmonary venous connections: a pictorial essay.

Anomalous pulmonary venous connections represent a heterogeneous group of congenital heart diseases in which a part or all pulmonary venous flow drains directly or indirectly into the right atrium. Clinically, anomalous pulmonary venous connections may be silent or have variable consequences, including neonatal cyanosis, volume overload and pulmonary arterial hypertension due to the left-to-right shunt. Anomalous pulmonary venous connections are frequently associated with other congenital cardiac defects and their accurate diagnosis is crucial for treatment planning. Therefore, multimodality diagnostic imaging, comprising a combination (but not all) of echocardiography, cardiac catheterization, cardiothoracic computed tomography and cardiac magnetic resonance imaging, helps identify potential blind spots relevant to each imaging modality before treatment and achieve optimal management and monitoring. For the same reasons, diagnostic imaging evaluation using a multimodality fashion should be used after treatment. Finally, those interpreting the images should be familiar with the various surgical approaches used to repair anomalous pulmonary venous connections and the common postoperative complications.

Identification of rapid progression of right ventricular functional measures using three-dimensional cardiac computed tomography after total surgical correction of tetralogy of Fallot.

OBJECTIVE: To identify subsets of patients with tetralogy of Fallot (TOF) after total surgical correction demonstrating the rapid progression of right ventricle (RV) functional measures using cardiac computed tomography (CT) ventricular volumetry. METHODS: Rapid or slow progression of RV functional measures was determined in 109 patients with TOF who underwent cardiac CT ventricular volumetry more than twice after total surgical correction. Patient age, body surface area, postoperative days, the time interval between the first and last cardiac CT examinations, and CT-based functional measures were evaluated using binary logistic regression to determine the predictors of the rapid progression. Receiver operating characteristic curve analysis was performed to evaluate diagnostic performance of the potential predictors. RESULTS: The rapid progression of indexed RV end-systolic volume (ESV) (≥2.7 mL/m2/year) and indexed RV end-diastolic volume (≥0.9 mL/m2/year) could be predicted by RV ejection fraction (EF) at the last cardiac CT with an odds ratio of 1.340 (95 % confidence interval [CI], 1.122-1.600; p = 0.001) and age at the last cardiac CT with an odds ratio of 8.255 (95 % CI, 1.531-44.513; p = 0.014), respectively. RV EF at the last cardiac CT showed the highest diagnostic performance (area under the curve = 0.799; p < 0.002) for the rapid progression of indexed RV ESV. CONCLUSION: Cardiac CT ventricular volumetry can be used to identify patients demonstrating the rapid progression of RV functional measures after total surgical correction of TOF and follow-up imaging protocols can be individually optimized based on initial progression rate.

Prediction of pulmonary hypertension using central-to-peripheral pulmonary vascular volume ratio on three-dimensional cardiothoracic CT in patients with congenital heart disease.

PURPOSE: To evaluate whether the central-to-peripheral pulmonary vascular volume ratio measured using three-dimensional cardiothoracic CT can serve as a potential predictor of pulmonary hypertension (PH) in patients with congenital heart disease. METHODS: Cardiothoracic CT was used to quantify right and left total, central, and peripheral pulmonary vascular volumes segmented based on a three-dimensional threshold-based approach in 60 patients with congenital heart disease (group with PH, n = 30; group without PH, n = 30). The CT-based central-to-peripheral pulmonary vascular volume ratios were correlated with the echocardiography-based maximum velocity of tricuspid regurgitation (TR Vmax) and systolic pulmonary arterial pressure (PAP) values. The diagnostic ability of the central-to-peripheral pulmonary vascular volume ratio to predict PH was analyzed. RESULTS: The central-to-peripheral pulmonary vascular volume ratios were significantly higher in the group with PH compared to the group without PH (1.6 ± 0.9 vs. 0.8 ± 0.3 for the right side, p < 0.001; 2.4 ± 2.2 vs. 1.0 ± 1.4 for the left side, p < 0.004). The right central-to-peripheral pulmonary volume ratios were significantly positively correlated with the TR Vmax and estimated systolic PAP values (R = 0.627 and 0.633, respectively; p < 0.001) in patients with and without PH, while the ratios were moderately correlated with the TR Vmax and estimated systolic PAP values (R = 0.431 and 0.435, respectively; p < 0.020) in the group with PH. The right and left central-to-peripheral pulmonary vascular volume ratios demonstrated a good diagnostic ability for predicting the presence of PH (area under the receiver-operating characteristic curve = 0.867, p < 0.001 and 0.859, p < 0.001 for the right and left, respectively). CONCLUSION: The CT-based central-to-peripheral pulmonary vascular volume ratio can be used to predict PH in patients with congenital heart disease.

Contrast-Enhanced CT Protocol for the Fontan Pathway: Comparison Between 1- and 3-Minute Scan Delays.

Optimal enhancement of the Fontan pathway is crucial for the accurate CT evaluation. Current guidelines for contrast-enhanced CT protocols are rather inconsistent in scan delays and injection methods. This single-center, retrospective study was performed to compare objective measures of contrast enhancement between 1- and 3-min scan delays (41 and 36 patients, respectively) to determine a better contrast-enhanced CT protocols for evaluating the Fontan pathway. In both groups, a biphasic injection protocol, in which 50% diluted contrast agent (the amount of iodinated contrast agent: 2.0 mL/kg; the amount of saline: 2.0 mL/kg) was injected at the injection rate of 0.5‒2.5 mL/s for 50 s followed by a saline flush at the same injection rate (0.5‒2.5 mL/s), was used. The degree and heterogeneity of cardiovascular enhancement, image noise, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) were quantitatively evaluated. The mean densities of all cardiovascular structures were significantly higher in the 1-min delay protocol than in the 3-min delay protocols (p < 0.001). Heterogeneous enhancement (normalized standard deviation > 0.70) in the Fontan pathway was significantly more frequent in the 1-min delay protocol (p < 0.001). No significant differences were found in image noise (p > 0.141) and the frequency showing suboptimal noise (p = 1.000) between the two protocols. SNR and CNR were significantly lower in the 3-min delay protocol (p < 0.001). Compared with the 1-min delay protocol, the 3-min delay protocol achieved more homogeneous enhancement in the Fontan pathway on CT but showed lower contrast enhancement, SNR, and, CNR, indicating the need for further improvement.

Diagnostic imaging for absent pulmonary valve syndrome: an update with an emphasis on cardiothoracic computed tomography.

Absent pulmonary valve syndrome is a rare congenital heart disease characterized by partial or complete absence of pulmonary valve cusps which commonly presents with respiratory difficulty during infancy. Because central airway compression by dilated central pulmonary arteries is a key pathology of this syndrome responsible for clinical presentation, severity, and outcome, cardiothoracic computed tomography (CT) is currently regarded as the imaging modality of choice before and after treatment. In addition, tracheobronchomalacia frequently responsible for persistent respiratory problems can be accurately evaluated with conventional two-dimensional cine CT or four-dimensional CT. In this pictorial review, various diagnostic imaging methods used to evaluate absent pulmonary valve syndrome are comprehensively illustrated with an emphasis on a recently spotlighted role of cardiothoracic CT.

Optimal end-systolic cardiac phase prediction for low-dose ECG-synchronized cardiac CT.

PURPOSE: To predict optimal end-systolic (ES) cardiac phase for low-dose ECG-synchronized cardiac computed tomography (CT). MATERIALS AND METHODS: ECG-synchronized ES cardiac CT examinations of 2441 patients from September 2010 to December 2016 were reviewed. Of them, 891 examinations acquired with an extended period of full tube current in a cardiac cycle (i.e., 10 % of RR interval or ≥100 ms) and adequate image quality (median patient age, 7 years; age range, 0 day‒60 years) were included. Absolute and relative delays (n = 861 and n = 30, respectively) of the cardiac CT were correlated with the heart rates. Best-fit equations were developed from the trend line with the highest coefficient of determination (R2) value for the two delays, and their success rates to obtain optimal ES phase in a padding with full tube current were calculated and compared with that of the T wave location method. CT radiation dose ratio was calculated as a width ratio of paddings with full tube current. RESULTS: The absolute and relative delays in the Pearson correlation test demonstrated a strong negative correlation (R = -0.9, p < 0.001) and a nearly moderate positive correlation (R = 0.5, p < 0.001) with heart rates, respectively. Two best-fit equations could be developed separately for both relative (R2 = 0.3) and absolute delays (R2 = 0.8). When adjusting the period of full tube current to a 114 ms for absolute delay and a 17.4 % of RR interval for relative delay, success rates of 94.9 % and 95.1 %, respectively, could be achieved and were significantly higher than that determined by the T wave location (82.7 %, p <  0.001). CONCLUSION: The best-fit equations method has a higher success rate for predicting the optimal end-systolic phase of ECG-synchronized cardiac CT than the T wave location method.

Double Outlet Right Ventricle: In-Depth Anatomic Review Using Three-Dimensional Cardiac CT Data.

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

Imaging Findings of Coronary Artery Fistula in Children: A Pictorial Review.

Coronary artery fistula, defined as an abnormal communication between the coronary arteries and a cardiac chamber (most commonly) or a thoracic great vessel, may result in hemodynamically significant problems due to vascular shunting in children. Echocardiography, cardiac catheterization, cardiac MRI, and cardiac CT may be used to evaluate coronary artery fistula in children. Recently, CT has played a pivotal role for the accurate diagnosis of coronary artery fistula in children. Surgical or interventional treatment is performed for hemodynamically significant coronary artery fistulas. In this pictorial review, the detailed imaging findings of coronary artery fistula in children are described.

Anomalous Origin of the Coronary Artery from the Pulmonary Artery in Children and Adults: A Pictorial Review of Cardiac Imaging Findings.

Anomalous origin of the coronary artery from the pulmonary artery is a rare and potentially fatal congenital heart defect. Up to 90% of infants with an anomaly involving the left coronary artery die within the first year of life if left untreated. Patients who survive beyond infancy are at risk of sudden cardiac death. Cardiac CT and MRI are increasingly being used for the accurate diagnosis of this anomaly for prompt surgical restoration of the dual coronary artery system. Moreover, life-long imaging surveillance after surgery is necessary for these patients. In this pictorial review, multimodal cardiac imaging findings of this rare and potentially fatal coronary artery anomaly are comprehensively discussed, and representative images are provided to facilitate the understanding of this anomaly.

Pediatric Cardiothoracic CT Guideline Provided by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group: Part 2. Contemporary Clinical Applications.

The use of pediatric cardiothoracic CT for congenital heart disease (CHD) was traditionally limited to the morphologic evaluation of the extracardiac thoracic vessels, lungs, and airways. Currently, the applications of CT have increased, owing to technological advancements in hardware and software as well as several dose-reduction measures. In the previously published part 1 of the guideline by the Asian Society of Cardiovascular Imaging Congenital Heart Disease Study Group, we reviewed the prerequisite technical knowledge for clinical applications in a user-friendly and vendor-specific manner. Herein, we present the second part of our guideline on contemporary clinical applications of pediatric cardiothoracic CT for CHD based on the consensus of experts from the Asian Society of Cardiovascular Imaging CHD Study Group. This guideline describes up-to-date clinical applications effectively in a systematic fashion.

Imaging findings of multisystem inflammatory syndrome in children associated with COVID-19.

BACKGROUND: A hyperinflammatory immune-mediated shock syndrome has been recognised in children exposed to the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the virus that causes coronavirus disease 2019 (COVID-19). OBJECTIVE: To describe typical imaging findings in children with multisystem inflammatory syndrome associated with COVID-19. MATERIALS AND METHODS: During the first wave of the COVID-19 pandemic, imaging studies and clinical data from children treated for multisystem inflammatory syndrome were collected from multiple centres. Standardised case templates including demographic, biochemical and imaging information were completed by participating centres and reviewed by paediatric radiologists and paediatricians. RESULTS: We included 37 children (21 boys; median age 8.0 years). Polymerase chain reaction (PCR) testing was positive for SARS-CoV-2 in 15/37 (41%) children and immunoglobulins in 13/19 children (68%). Common clinical presentations were fever (100%), abdominal pain (68%), rash (54%), conjunctivitis (38%) and cough (32%). Thirty-three children (89%) showed laboratory or imaging findings of cardiac involvement. Thirty of the 37 children (81%) required admission to the intensive care unit, with good recovery in all cases. Chest radiographs demonstrated cardiomegaly in 54% and signs of pulmonary venous hypertension/congestion in 73%. The most common chest CT abnormalities were ground-glass and interstitial opacities (83%), airspace consolidation (58%), pleural effusion (58%) and bronchial wall thickening (42%). Echocardiography revealed impaired cardiac function in half of cases (51%) and coronary artery abnormalities in 14%. Cardiac MRI showed myocardial oedema in 58%, pericardial effusion in 42% and decreased left ventricular function in 25%. Twenty children required imaging for abdominal symptoms, the commonest abnormalities being free fluid (71%) and terminal ileum wall thickening (57%). Twelve children underwent brain imaging, showing abnormalities in two cases. CONCLUSION: Children with multisystem inflammatory syndrome showed pulmonary, cardiac, abdominal and brain imaging findings, reflecting the multisystem inflammatory disease. Awareness of the imaging features of this disease is important for early diagnosis and treatment.

Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia.

BACKGROUND: To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors. METHODS: Children with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed. RESULTS: Of the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25-75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume. CONCLUSIONS: A quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV. .