Outcome of Tricuspid Valve Plasty in Norwood Stage I Operation.

BACKGROUND: Significant tricuspid valve regurgitation (TR) is considered a poor prognosis factor for patients with hypoplastic left heart syndrome (HLHS). Performing a tricuspid valve plasty (TVP) during Norwood Stage I palliation (S1P) has rarely been reported. We report mid-term results of performing TVP during Norwood S1P. METHODS AND RESULTS: Between December 2004 and July 2013, 48 patients with HLHS or variants underwent Norwood S1P and of them 11 (23%) with TR of a moderate degree or above underwent TVP. The estimated 1- and 5-year survival rates for Norwood S1P were 61% and 54%, respectively. Among the 11 patients with TVP, there were 2 early deaths. Nine patients had a Stage II operation and 3 of them died late. Four patients completed a Fontan operation, and 2 were waiting. Using Cox regression analysis, lower body weight, presence of intact atrial septum, and preoperative cardiopulmonary resuscitation were factors associated with increased risk for death. Patients with significant TR and undergoing TVP during Norwood S1P had a similar survival curve to those without significant TR. CONCLUSIONS: Our results for TVP performed during Norwood S1P were encouraging. They suggested that aggressive TVP is warranted in Norwood S1P when primary heart transplantation is not available. Further study is required to determine if the strategy does improve the results for patients with HLHS and TR initially. (Circ J 2016; 80: 1362-1370).

The Concept of the Arch Window in the Spiral Switch of the Great Arteries.

When the arterial switch operation includes the Lecompte maneuver, the arterial trunks are reconnected in parallel, rather than the spiral fashion observed in the normal heart. Thus, although the ventriculo-arterial connections are hemodynamically corrected, the anatomic arrangement cannot be considered normal. We hypothesized that, if feasible, it would be advantageous to restore a spiral configuration for the arterial trunks. In 58 patients, we reconstructed the arterial trunks such that, postoperatively, the pulmonary channel spirals round the aorta, passing to either the right or the left, and branches posteriorly. We compared the outcomes with those in 95 patients undergoing a standard non-spiraling operation over the same period. Average follow-up was 8.2 ± 4.5 years. The estimated 10-year survival was similar in the cohorts, at 94.7 % for those with spiraling trunks, as compared to 90.4 % for those with parallel outflow tracts. Reoperation-free survival at 10 years was not significantly different (87.6 vs. 90.5 %). Supravalvar pulmonary stenosis, aortic neo-coarctation, or left bronchial stenosis, however, was encountered in one-eighth of those undergoing a standard operation. None of these complications occurred in those patients who, postoperatively, had spiraling outflow tracts (P = 0.002). Reconstruction of spiraling trunks after the arterial switch has, thus far, avoided the complications of supravalvar pulmonary stenosis, neo-aortic kinking, or bronchial stenosis. The spiraling arrangement prevents compression of the pulmonary vessels and bronchial tree by the aorta, since it provides a wide window in the new aortic arch.

Ventricular geometric characteristics and functional benefit of mild right ventricular outflow tract obstruction in patients with significant pulmonary regurgitation after repair of tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) outflow tract obstruction (RVOTO) might protect the RV from adverse remodeling caused by significant pulmonary regurgitation (PR) in patients with repaired tetralogy of Fallot (rTOF), but the underlying mechanisms and influences on exercise tolerance remain unclear. This study sought to investigate the impacts from mild RVOTO on ventricular remodeling and exercise capacity in rTOF. METHODS: Eighty-five rTOF patients with a PR fraction ≥20% were assessed with cardiac magnetic resonance, cardiopulmonary exercise test, and echocardiography. Patients with a peak RVOT pressure gradient 20-50 mmHg were considered to have mild RVOTO (n = 29), while those with a gradient <20 mmHg had isolated PR (n = 56). RESULTS: Comparing to patients with isolated PR, patients with combined PR and mild RVOTO had smaller RV and RVOT dimension, better RV and left ventricular (LV) ejection fraction (EF), and superior exercise capacity. PR severity and RV mass/volume ratio were similar between these 2 groups. LVEF coupled with RVEF only in patients with isolated PR. In multivariate analysis, smaller RVOT dimension was independently related to smaller RV dimension (P < .001) and higher RVEF (P = .005). Furthermore, mild RVOTO was independently associated with higher peak oxygen consumption (P = .014) and oxygen uptake efficiency slope (P = .005). CONCLUSIONS: Patients with combined PR and mild RVOTO had better RV remodeling and exercise capacity compared to those with isolated PR. Our findings confirm the benefits from mild residual RVOTO support a policy of conservative RVOTO relief at repair.

Risk stratification and outcome of cardiac surgery for patients with body weight <2 ,500g in an Asian center.

BACKGROUND: Cardiac surgery performed in patients with low body weight is a challenge for surgeons. Currently, such outcomes are mainly reported from European or North American centers. In this study, we review our cardiac surgery experience with neonates and infants weighing <2,500g. METHODS AND RESULTS: We included patients with a body weight <2,500g who received cardiac surgery between January 2008 and December 2012. The survival outcome was compared to that of patients with large body weight, and then the Risk Adjusted Classification for Congenital Heart Surgery (RACHS-1) categorization was used for operative risk stratification. In the 1,245 index operations, 53 patients (4.3%) were <2,500g. The mean body weight was 2,232g (range 1,320-2,500g). The hospital mortality rate was 20.7% (11/53). Most (85%) of the procedures were in RACHS-1 category ≥3. The risk ratio was significantly higher in RACHS-1 category 3 (relative risk [RR]:6.2; 95% confidence interval [CI]:1.6-23.9) and 4 (RR:4.6; 95% CI:1.4-15.0), respectively, while it was not significantly different in category 2 (RR:1.02; 95% CI:1.01-1.02) and category 6 (RR:2.9; 95% CI:0.36-13.3). CONCLUSIONS: Cardiac surgery performed on infants with low body weight is generally a complex procedure, but the results are acceptable. The risk was higher than that for patients with higher body weight in RACHS-1 category 3 and 4. Further investigation to improve the outcome of this high-risk group is needed.

Comparison of bilateral pulmonary arterial level and diameter in transposition of the great arteries.

In normal anatomy, the left pulmonary artery (LPA) is usually situated higher than the right pulmonary artery (RPA); however, transposition of the great arteries (TGA), the LPA is not always situated higher than the RPA. This study was performed to clarify the relative position of the RPA and the LPA in transposition of the great arteries (TGA) as well as the implications. We reviewed 101 angiograms of patients with TGA (age 4.1 ± 1.2 months). The width of the RPA, the LPA, and the pulmonary trunk (PT) were measured just before their first branch in the frontal view. They were classified into four groups according to the ratio between the RPA and the PT (RPA/PT). The initial courses of the LPA and the RPA were compared and defined according to their height in the frontal view, and the preferential flow (or not) to the RPA was recorded. The equation of hydrodynamics was applied to evaluate the bifurcation angle. Both PAs were the same size in all cases. Forty-eight patients (47.5 %) had a RPA/PT diameter ratio < 0.49. The LPA coursed higher than the RPA in the majority of cases (81 [80.2 %]); in a minority of cases the LPA and RPA were at the same level (6 [5.9 %]); and in some cases the RPA coursed higher than the LPA (14 [13.9 %]). Patients with a high degree of PA hypoplasia tended to have both PAs at the same level or a higher-positioned RPA. Autopsy (1 of 3 cases) showed a posterior ridge against the bronchus in the higher RPA. Hydrodynamic calculation showed that the greater the angle between the RPA/PT, the greater the preferential flow. Preferential flow to the RPA in TGA did not necessarily result in LPA hypoplasia before its first branch. Higher RPA position relative to the LPA was associated with greater flow in it against the posterior bronchus. This situation was more prevalent in patients with severe PA hypoplasia.

Two-stage endovascular balloon angioplasty and stent implantation for supravalvular pulmonary stenosis in a 33-month-old boy incurring congestive heart failure following arterial switch operation.

We report a 33-month-old boy, who had undergone arterial switch operation (ASO) for simple transposition of the great arteries (TGA) since birth, presenting with exercise intolerance with dyspnoea, tachypnoea, tachycardia, cardiomegaly and hepatomegaly. Doppler echocardiography showed severe tricuspid regurgitation and dilatation of the right atrium. At 28 months, cardiac catheterization tracked down the culprit to be right ventricular outflow tract obstruction (RVOTO) manifested at the mid-portion of the main pulmonary artery (MPA) as supravalvular pulmonary stenosis (SVPS) and measuring 2.98 mm in diameter. There was a pressure gradient of 76 mmHg between the right ventricle and the distal portion of the MPA. The lumen was initially dilated to 6.79 mm by balloon angioplasty (BA), after which the pressure gradient dropped to 51 mmHg. However, cardiomegaly and hepatomegaly lingered for 5 months. Stent implantation (SI) was performed at 33 months, after which the lumen was dilated to 9.99 mm and the pressure gradient dropped to 20 mmHg. Liver span decreased from 10 cm to 6 cm.There was a recrudescence of physical activity. Echocardiography showed regression of tricuspid regurgitation and dilatation of the right atrium at the 12-month follow-up.

Long-Term Outcomes of Native Coarctation of the Aorta after Balloon Angioplasty or Surgical Aortoplasty in Newborns and Young Infants Less Than 3 Months of Age.

BACKGROUND: Balloon angioplasty of native coarctation of the aorta, though effective, is frequently associated with early restenosis, repeated interventions and aneurysm formation, especially in very young patients. However, available long-term data are limited. METHODS: From Jan. 1986 to Dec. 2007, eighty-eight patients with simple aortic coarctation, isolated or associated with patent ductus arteriosus or ventricular septal defect, and survived angioplasty (n = 17) or surgery (n = 71) within 3 months of age in National Taiwan University Hospital were enrolled. RESULTS: The average length of follow-up was 10.1 ± 5.1 years. The long-term outcomes between these two groups were comparable in hypertension, left ventricular outflow tract obstruction, and aortic aneurysm. Patients in the angioplasty group had a greater likelihood of reintervention (64.7% vs. 42.3%, p = 0.096) and repeated reintervention (29.4% vs. 7.0%, p = 0.027) as compared with surgery group. The 10-year freedom from reintervention was 35.3% and 59.2% in the angioplasty and surgery group, respectively (p = 0.046); there were no deaths reported in that 10-year span. Patients with angioplasty and those with patent ductus arteriosus were more likely to require surgical aortoplasty at reintervention. CONCLUSIONS: The long-term outcomes of aortic coarctation in newborns and young infants after balloon angioplasty were effective and beneficial. However, those outcomes were accompanied by concomitant higher risks of reintervention and surgical reintervention, particularly in those with patent ductus arteriosus. KEY WORDS: Aortic operation; Coarctation; Infant; Outcomes.

Detection of pulmonary arterial morphology in tetralogy of Fallot with pulmonary atresia by computed tomography: 12 years of experience.

UNLABELLED: Our aim was to evaluate the feasibility of using computed tomography (CT) to define the pulmonary artery anatomy in patients with tetralogy of Fallot and pulmonary atresia (TOF-PA). We retrospectively reviewed 110 patients with TOF-PA between 1995 and 2008. Those who received cardiac catheterization and surgery within 3 months of their CT examinations were enrolled. Based on Dr. Somerville's classification, the pulmonary arterial pattern was determined, including identifiable pulmonary trunk (type I), the presence of both left and right pulmonary arteries without trunk (II), only left or right pulmonary artery present (III), and absent intrapericardial pulmonary arteries (IV). The accuracy of both imaging modalities was evaluated with operation findings as the golden standard. The effective radiation doses and adverse events were also recorded. In the 64 eligible patients (median age, 23 months), CT and catheterization demonstrated accurate pulmonary arterial morphology in 60 (60/64) and 53 (53/64) TOF-PA patients, respectively. Thirty-two of 35 type I patients were correctly identified by CT, whereas 26 were correctly identified by catheterization (p = 0.03). Of the 20 type II TOF-PA patients, 19 were diagnosed by CT, whereas 18 were diagnosed by catheterization. CT and catheterization both successfully defined six type III and three type IV patients. The median calculated radiation doses caused by CT and catheterization were 4.5 and 5.6 mSv, respectively (p > 0.05). CONCLUSIONS: For patients with TOF-PA, CT could accurately delineate pulmonary arterial morphology with the same level of accuracy as cardiac catheterization. Therefore, CT can be considered a reasonable diagnostic alternative for such patients.

Natural and unnatural history of tetralogy of Fallot repaired during adolescence and adulthood.

Surgical repair of tetralogy of Fallot (TOF) in countries with sound medical care systems is seldom delayed until adolescence. This study investigated the clinical profile and the surgical outcomes in such a population from Taiwan. Between 1970 and 2009, 179 TOF patients (56% male) received total repair at 19.2 ± 8.3 (10-49) years of age. We reviewed the medical records and interviewed the patients concerning their current status. The survival was ascertained in all by a link to our national health database. Major morbidities before cardiac repair included atrial arrhythmia (1.1%), ventricular arrhythmia (3.9%), infective endocarditis (6.7%), brain abscess (4.6%) and pulmonary tuberculosis (3.3%). Ventricular arrhythmia and pulmonary tuberculosis occurred mainly after 20 years of age. Thirty patients (16.8%) received a palliative shunt. The preoperative QRS duration increment was 0.6 ms/year. Early mortality occurred in 4 (2.2%) and was related to previous shunt surgery (OR = 16.5, p < 0.05) and coronary artery crossing RVOT (OR 17.6, p < 0.05). After repair, the functional class improved in all patients. The median age at latest follow-up was 31.8 (32.8 ± 12.3) years. The survival was 92.7 and 89.3% at 20 and 30 years after operation, respectively. Late cardiovascular death could be predicted by the length of postoperative intensive care unit stay (OR = 1.3, p < 0.001). The freedom from ventricular arrhythmia 30 years after repair was 84.1% and was associated with a final QRS longer than 160 ms. Unrepaired TOF patients were at high risk of infective endocarditis, brain abscess, pulmonary tuberculosis and arrhythmias during their adolescence and adulthood. Cardiac repair in this age group was still safe and effective.

Understanding coronary arterial anatomy in the congenitally malformed heart.

With the development of three-dimensional techniques for imaging, such as computed tomography and magnetic resonance imaging, it is now possible to demonstrate the precise sinusal origin and epicardial course of the coronary arteries with just as much accuracy as can be achieved by the morphologist holding the heart in his or her hands. At present, however, there is no universally accepted convention for categorising the various patterns found when the heart is congenitally malformed. In this review, we show how, to provide such a convention, it is necessary to take note not only of the sinusal origin of the three major coronary arteries, but also the relationship of the aortic root relative to the cardiac base. We summarise the evidence showing how the proximal portions of the developing coronary arteries grow into the aortic valvar sinuses subsequent to the separation of the aortic root from the subpulmonary infundibulum. We also discuss the evidence showing that the subpulmonary myocardium is impervious to the passage of epicardial coronary arteries, and suggest that the process of septation itself plays an integral role in guiding the arteries into the two aortic sinuses that are adjacent to the pulmonary root. We then show how marriage of convenience between the epicardial coronary arteries and the aortic valvar sinuses provides a good explanation for the known variations found in the setting of transposition. We point out that it is the absence of septation that likely governs the patterns seen in the setting of a common arterial trunk.

Coronary artery anatomy in complete transposition with situs solitus and dextrocardia.

The coronary artery anatomy of complete transposition with situs solitus/levocardia (CTSSL) has been well elucidated in the current era of arterial switch operation. However, coronary artery for complete transposition with situs solitus/dextrocardia (CTSSD) has never been documented. Coronary anatomy of transposition and aortopulmonary rotation were identified by angiography or surgical intervention from 1988 to 2007 at our hospital. The degree of aortopulmonary rotation was defined by the aortic sinus pattern on lateral angiogram. Apicocaval ipsilaterality was defined as situs solitus/dextrocardia or situs inversus/levocardia. The coronary artery anatomy in 3 cases of CTSSD was analyzed and correlated with those patients having transposition with the same coronary pattern but without apicocaval ipsilaterality, i.e., 276 cases with CTSSL and 8 cases with complete transposition with situs inversus/dextrocardia (CTSID). Fisher's exact test was used to determine statistical significance. All three cases with CTSSD (with apicocaval ipsilaterality) had a single coronary artery piercing into the left-hand sinus with a right coronary artery in the posterior atrioventricular groove, whereas all 284 cases without apicocaval ipsilaterality (CTSSL or CTSID) had the left circumflex artery in the posterior atrioventricular groove. The aorta was significantly less left laterally rotated in CTSSD than the other 2 cases of CTSSL and 3 cases of CTSSD with a similar coronary pattern (p < 0.05). One may anticipate coronary artery anatomy in the posterior atrioventricular groove based on apicocaval ipsilaterality, which in turn decreases aortopulmonary rotation to predict the central coronary pattern.

Staged surgical repair for infants with interrupted aortic arch.

BACKGROUND: Interrupted aortic arch (IAA) is a rare congenital cardiac anomaly, which necessitates surgical treatment. There are several surgical strategies for corrective repair of IAA, such as one-stage repair, rapid two-stage repair and two-stage repair. Here, we reported our surgical result of staged-repair policy for the patients with IAA. METHOD: From November 2003 to July 2015, there were 14 patients (8 boys, 6 girls) with IAA treated by us. Except one teenager patient, we routinely used intravenous infusion of prostaglandin E1 for all the infant patients (n = 13) to keep adequate end organ perfusion before the first surgical intervention. Surgical repair was performed after the perfusion of end organs recovered. RESULT: Two patients (1 teenager and 1 infant with one-stage surgery) were excluded from this study. At the time of the first surgery, we did the first-stage surgery with anastomosis in between aortic arch and descending aorta, division of patent ductus arteriosus and banding of pulmonary trunk through left thoracotomy. The overall surgical survival rate of the first surgery was 100% (12/12). At the time of the second surgery, corrective repair was done under cardiopulmonary bypass through median sternotomy. The surgical survival rate of the corrective surgery was also 100%. There is no late death during follow-up for 9 years (range 4.2-15.0 years). CONCLUSION: Out of several surgical strategies for the infants with IAA, staged repair still could be a treatment option to achieve satisfied surgical result.

Clinical spectrum and long-term outcome of Ebstein's anomaly based on a 26-year experience in an Asian cohort.

Ebstein's anomaly is a rare, congenital cardiac anomaly that may result in cyanosis, right heart failure, and tachyarrhythmia during the newborn stage or after adolescence. This study investigated the data of 77 patients diagnosed between 1980 and 2006 at a tertiary care center in Taiwan. Patients were grouped into either an early group or a late group. Survival declined rapidly within the newborn stage in the early group, but declined only during the third decade in the late group. Surgical results were poor (20% success rate) for neonatal systemic-to-pulmonary shunts in those cases with associated pulmonary atresia, but were satisfactory for other surgical modes. Supraventricular tachyarrhythmia occurred in 31 (41%) patients at a median age of 10 years and could be eliminated by radiofrequency ablation (81% success rate), though the recurrence rate was high (41%). In conclusion, other than those cases requiring shunts at the newborn stage, the long-term outcome was favorable.

Surgical ligation of patent ductus arteriosus in very-low-birth-weight premature infants in the neonatal intensive care unit.

This study reported our experience of bedside patent ductus arteriosus (PDA) ligation for prematurity in the neonatal intensive care unit (NICU). Between April 1992 and March 2006, 41 very-low-birth-weight premature infants underwent PDA ligation in the NICU. There were 18 male and 23 female infants. The mean gestational age and birth weight were 26.9 weeks and 900.9 g, respectively. Preoperatively, 25 infants were ventilator-dependent. After operation, there were five deaths caused by complications of prematurity. Surgical complications occurred in four and all recovered well after treatment. Twenty preoperatively intubated babies survived and were extubated at 21.6 +/- 12.7 days postoperatively. In conclusion, bedside PDA ligation in the NICU is safe and effective. It can avoid transportation of critically ill, very small infants. We suggest surgical closure as the primary treatment in very-low-birth-weight infants who are ventilator-dependent to avoid the possible complications of indomethacin and prolonged intubation.

Modeling torsion of blood vessels in surgical simulation and planning.

This paper proposes a hybrid approach for modeling torsion of blood vessels that undergo deformation and joining. The proposed model takes 3D mesh of the blood vessel as input. It first fits a generalized cylinder to extract the blood vessel's medial axis. Then, it uses rotation minimizing frame as a reference to model and measure the torsion of blood vessel after deformation. In general, the proposed approach can incorporate any kind of deformation algorithms. In our experiments, differential geometry method is used as an example. The test results show that our algorithm can correctly and effectively evaluate the amount of torsion caused by blood vessel deformation. In addition, it can also determine the configuration of the blood vessel with minimum torsion.

Predictive surgical simulation of aorta reconstruction in cardiac surgery.

This paper proposes a method for performing predictive simulation of complex cardiac surgery. It computes complex surgical results given a small amount of user inputs. In this way, the surgeon can easily explore various surgical options without having to go through all the detailed steps of the surgical procedure. Test results, using aorta reconstruction as an application example, show that the proposed method can generate realistic simulation results given different kinds of user inputs, thus demonstrating the feasibility of the approach.

Extracorporeal membrane oxygenation rescue for cardiopulmonary resuscitation in pediatric patients.

OBJECTIVE: To describe survival and neurologic outcome and identify the factors associated with survival among pediatric patients following extracorporeal cardiopulmonary resuscitation (ECPR) for in-hospital cardiac arrest. DESIGN: Retrospective study. SETTING: A university-affiliated tertiary care hospital. PATIENTS: Eligible patients were < or = 18 yrs of age and received extracorporeal membrane oxygenation during active cardiopulmonary resuscitation for in-hospital cardiac arrest. INTERVENTIONS: Extracorporeal membrane oxygenation (ECMO) during active cardiopulmonary resuscitation. MEASUREMENTS AND MAIN RESULTS: The primary outcome was survival to hospital discharge. The secondary outcome was neurologic status after ECPR at hospital discharge and late follow-up. Good neurologic outcome was defined as Pediatric Cerebral Performance Categories 1, 2, and 3. Continuous variables were expressed as medians (interquartile range). We prospectively defined the early cohort (January 1999 to December 2001) and late cohort (January 2002 to January 2006) and compared the survival rates. We identified 27 ECPR events. The survival rate to hospital discharge was 41% (11 of 27). The nonsurvivors had higher pre-cardiopulmonary resuscitation serum lactate levels (14 [10.2-19.6] mmol/L vs. 8.5 [4.4-12.6] mmol/L, p < .01), longer durations of cardiopulmonary resuscitation (60 [37-81] mins vs. 45 [25-50] mins, p < .05) with longer activating time for ECMO (12.5 [7.5-33.8] mins vs. 5 [0-10] mins, p < .01), and more renal failure after ECPR (68% [11 of 16] vs. 9% [1 of 11], p < .01). The survival rate of the late cohort was better than that of the early cohort (58% [11 of 19] vs. 0% [0 of 8], p < .05). By exact multiple logistic regression analysis, the early cohort and renal failure after ECPR were two independent risk factors for mortality. Among the 11 survivors, ten had good neurologic outcomes. CONCLUSIONS: ECPR successfully rescued some pediatric patients who failed rescue with conventional in-hospital CPR. Good neurologic outcomes were achieved in the majority of the survivors. Early cohort and post-ECPR renal failure were associated with mortality.

Prediction of early pulmonary artery stenosis after arterial switch operation: the role of intraoperative transesophageal echocardiography.

OBJECTIVES: To investigate prospectively the prediction of the neopulmonary stenosis (neo-PS) after arterial switch operation (ASO) for transposition of the great artery (TGA) with intraoperative transesophageal echocardiography (TEE). METHODS: Infants with TGA undergoing the ASO were prospectively studied over 5 years. The neo-PS was defined when the peak flow velocity was over 3 m/s at the neo main pulmonary artery (neo-MPA) after ASO by TEE (TEEPS). Catheterization was performed if estimated peak neo-PS pressure gradient was over 40 mm Hg by transthoracic echocardiography. Balloon angioplasty was tried first and surgical reoperation was reserved for those with failed angioplasty. RESULTS: In total 49 consecutive patients were enrolled into the cohort study. TEEPS was identified in 21 patients. For patients with TEEPS, freedom from reintervention was 28% at 1 year and 23% at 2 years. For patients without TEEPS, freedom from reintervention for PS was 92% at 1 year and 78% at 2 years. The time interval from ASO to reintervention was significantly shorter in patients with TEEPS than without TEEPS. Existence of TEEPS and non-Lecompte method were main risk factor for reintervention. CONCLUSION: The present study demonstrated that the application of intraoperative TEE for infants undergoing ASO is very helpful in predicting the development of early postoperative neo-PS.

A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system.

PURPOSE: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM). MATERIALS AND METHODS: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly. RESULTS: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine. CONCLUSION: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.

Crossed pulmonary arteries: report of two cases with emphasis on three-dimensional helical computed tomographic imaging.

Crossed pulmonary arteries are due to an anomalous origin of both pulmonary arteries from the main pulmonary trunk. This anatomy is often associated with other congenital cardiac and extracardiac diseases. We report two neonates with complex congenital heart disease who had this disorder, which was detected during cardiac computed tomography (CT) with three-dimensional reconstruction but not during echocardiography or angiography. The first patient was a 3-day-old male neonate who had tachypnea and feeding problems since birth. Cardiac CT showed crossed pulmonary arteries, type B interruption of the aortic arch, a ventricular septal defect, and a large patent ductus arteriosus. He received an emergency T-colostomy at 3 days of age because of severe bowel distention. Low-type imperforated anus was diagnosed. His postoperative course was complicated with fluctuated saturation, seizure, hypocalcemia, hyperphosphatemia, and sepsis. Also found were cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia, and a variable deletion on chromosome 22q11 (CATCH 22 disorder). Because of his poor prognosis, the patient was transferred to another hospital on day 16 for further care, at the family's request. The other patient was a 5-day-old female neonate who had a heart murmur since birth. Cardiac CT showed crossed pulmonary arteries, truncus arteriosus, type A interruption of the aortic arch, a ventricular septal defect, an atrial septal defect, and a large patent ductus arteriosus. She received complete surgical correction, including division of the patent ductus arteriosus and repair of the other defects. Intermittent respiratory distress and decreased blood pressure complicated her postoperative course, and she died on the eighth day after surgery. Crossed pulmonary arteries complicated accurate interpretation of two-dimensional echocardiographs of the great vessels, as well as the course and location of catheters during cardiac catheterization. Three-dimensional CT provided a noninvasive approach to clearly recognize these malformations and the related anatomic structures. This information is important in planning and performing surgery in neonates with crossed pulmonary arteries.