Source Of Pulmonary Blood Supply Research Articles

Clarifying the Pulmonary Arterial Morphology and Pulmonary Blood Supply in Patients with Tetralogy of Fallot and Pulmonary Atresia on Computed Tomography Angiography

Aim. The present study sought to characterize the pulmonary arterial morphology and pulmonary blood supply in patients of tetralogy of Fallot and pulmonary atresia (TOF-PA) on CT angiography. Materials and Methods. We retrospectively reviewed our departmental database to identify patients with TOF-PA evaluated using CT angiography. The images were analysed to detect the presence of main and branch pulmonary arteries and pulmonary arterial confluence, presence of major aortopulmonary collateral arteries (MAPCAs), laterality and relation with pulmonary arterial morphology, and presence of patent arterial duct and associated intra- and extracardiac anomalies. Results. TOF-PA was identified in 177 patients (114 (64.4%) males; median age: 9 months). Pulmonary arteries were confluent in 142/177 (80.2%) patients. According to Somerville classification, type II pulmonary atresia was the most frequent pattern seen in 127/177 (71.8%). Based on McGoon’s ratio, pulmonary arteries were adequate for surgery in 123/177 (69.5%) patients. Patent arterial duct was present in 84/177 (47.5%) patients while MAPCAs were present in 124 (70.1%) patients, of which 72/124 (58.1%) patients had at least 1 essential MAPCA supplying either lung. According to Congenital Heart Surgeons’ Society classification, type B pulmonary arterial anatomy was the most prevalent, seen in 103/177 (57.6%) patients. Conclusion. TOF-PA is associated with marked morphologic variability in the pulmonary arterial arborization to supply the lungs. Cardiac CT angiography can accurately delineate the pulmonary arterial morphology, sources of pulmonary blood supply, and associated cardiovascular anomalies in patients with TOF-PA which aids in planning appropriate surgical management including decisions regarding the need for unifocalization of MAPCAs.

Pulmonary atresia and ventricular septal defect: How accurate is the fetal echocardiography, and do the major aortopulmonary collateral arteries matter?

To assess the accuracy of prenatal echocardiography in defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD). The second aim is to compare the perinatal and postnatal outcomes of different pulmonary blood supply types. The cases prenatally diagnosed with PAVSD between 2017 and 2022 in a single tertiary fetal medicine center were identified on the electronic database. Fetal echocardiography reports and images were reviewed retrospectively. Postnatal outcomes were acquired from the hospital records of relevant pediatric cardiology and cardiovascular surgery clinics. Fetal echocardiography results were compared with postnatal results. Perinatal and postnatal outcomes were compared between the different pulmonary vascular supply types. Among the 24 PAVSD cases, six were diagnosed with major aortopulmonary collateral arteries (MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA) dependent pulmonary supply, and seven were diagnosed with double pulmonary supply (MAPCA + DA) on prenatal echocardiography. Seventeen cases were live-born and have undergone postnatal investigations. Fetal echocardiography was 88.2% accurate about the type of pulmonary supply. The accuracy of fetal echocardiography regarding pulmonary vascular anatomy was 82.3%. Postoperative survival was 69.2%. Mortality before surgery and postoperative survival did not differ between pulmonary supply groups. Survival was impaired by the extracardiac anomalies. The need for early interventions was significantly higher in the DA group. Pulmonary vascularization in PAVSD can be defined precisely on fetal echocardiography. The source of pulmonary blood supply does not impact postnatal short-term outcomes significantly but it impacts the management. The associated anomalies highly contribute to postnatal mortality.

P1727 Large coronary artery to pulmonary artery fistula as primary source of pulmonary blood supply in tetralogy of fallot with pulmonary atresia

Abstract Funding Acknowledgements Not Applicable OnBehalf Not Applicable Introduction Pulmonary blood supply in patients of Tetralogy of Fallot with pulmonary atresia is usually from patent arterial duct or major aortopulmonary collaterals (MAPCAs) arising from descending thoracic aorta. We describe a case in which large coronary to pulmonary artery fistula was the primary source of pulmonary blood supply. Case Report A 17 years old female was referred to our hospital for diagnostic workup of suspected congenital heart disease. She was previously undiagnosed and now complains of progressive shortness of breath for last few months. On physical examination she was non-dysmorphic with oxygen saturation of ∼ 77 % in room air, blood pressure of ∼ 117/72 mmHg, pulse rate of ∼ 89 beats per minute and respiratory rate of ∼ 24 breaths per minute. She was clinically cyanosed with grade 3 clubbing and polycythemic. Cardiovascular examination revealed quiet precordium with normally placed apex beat, grade 2 parasternal heave with single second heart sound and grade 3/6 continuous murmur along left mid sternal border. Twelve lead electrocardiogram (ECG) showed normal sinus rhythm, right axis deviation and right ventricular hypertrophy. There was no evidence of ischemia. Chest X-ray revealed "boat shaped heart" with oligaemic lung fields. Transthoracic echocardiography showed large conoventricular ventricular septal defect with bidirectional flow. There was aortic over-ride with dilated left main coronary artery. No forward flow was seen across right ventricular outflow tract. Considering hugely dilated left main coronary artery, suspicion of coronary to pulmonary artery fistula was made and cardiac computed tomography followed by conventional angiography was done, both confirmed the diagnosis of Tetralogy of Fallot with pulmonary atresia and large coronary artery to main pulmonary artery fistula as a primary pulmonary blood supply. Two small collaterals (MAPCAs) were also identified supplying small part of right and left lungs. Conclusion This case highlights unusual source of pulmonary blood supply in Tetralogy of Fallot with pulmonary atresia. Correct pre-operative diagnosis is essential for appropriate surgical planning and better outcome. Abstract P1727 Figure. TOF-PA with CA to PA Fistula

Second stage after initial hybrid palliation for hypoplastic left heart syndrome: Arterial or venous shunt?

ObjectiveHybrid palliation for hypoplastic left heart syndrome has been developed as an alternative to neonatal Norwood surgery. At the second stage, a source of pulmonary blood flow has to be established. Options include an arterial modified Blalock–Taussig or a venous superior cavopulmonary shunt. MethodsWe retrospectively reviewed patients who received second-stage palliation after the initial hybrid. Patients were stratified according to the source of pulmonary blood supply into the arterial shunt (n = 17 patients) or venous shunt (n = 26 patients). ResultsAge and weight at second stage were lower in the arterial group (85 [45-268] days vs 152.5 [61-496] days, P = .001 and 3.6 [2.7-9.4] kg vs 5.1 [2.97-9.4] kg, P = .001, respectively). All recorded surgical times were shorter in the arterial group. Mechanical ventilation and intensive care stay were shorter in the venous group (5.82 [2.01-14.9] days vs 2.42 [0.56-13.67] days, P = .005 and 8.5 [3.6-23.7] vs 5.75 [0.8-17.6] days, P = .036, respectively) There was no difference in mortality (2/17 vs 5/26; P = .685) or incidence of complications between the 2 groups. There was a tendency toward a higher need for intervention in the immediate postoperative period in the venous group, but this did not reach significance (6/17 vs 13/26, P = .342). The arterial group has shown better development of the branch pulmonary arteries with a higher lower lobe index (158.38 ± 39.43 mm2/m2 vs 113.33 ± 43.96 mm2/m2, respectively, P = .037). ConclusionsBoth arterial and venous shunts are viable options with mortality and morbidity results comparable to those in the literature. The arterial shunt pathway (2-stage Norwood I) may offer better pulmonary arterial growth than the venous shunt (comprehensive/combined Norwood I and II).

Comparison of Contrast Enhanced Magnetic Resonance Angiography with Invasive Cardiac Catheterization for Evaluation of Children with Pulmonary Atresia

Complete assessment of the source of pulmonary blood supply and delineation of the anatomy of pulmonary arteries are essential for the management and prognostic evaluation of pulmonary atresia (PA) patients. Invasive cardiac catheterization is considered the gold standard imaging modality to achieve this. We investigated the role of contrast enhanced magnetic resonance angiography (MRA) to evaluate the pulmonary blood supply and the anatomy of the pulmonary arteries and compared this with cardiac catheterization in children with PA. We studied 20 children with PA. Median age was 2.5 years (range 6 months–13 years). All patients were examined with cardiac catheterization and contrast enhanced MRA, and the results of both modalities were compared. There was a complete agreement between both modalities in the detection of the main pulmonary artery morphology and determination of the confluence state of the central pulmonary arteries. There was an 88% agreement for patency of the ductus arteriosus and 66% for patency of the surgically placed shunt. There was a complete agreement between both techniques on determining the presence of collaterals more than 2.5 mm. Twenty-eight collaterals of less than 2.5 mm were detected only by contrast enhanced MRA. There was a strong correlation between both modalities in measuring the pulmonary arteries and collaterals diameter (P<0.001). Contrast enhanced MRA is a safe and accurate non-invasive technique to evaluate the pulmonary artery morphology and the sources of pulmonary blood supply in children with PA.

Gadolinium-Enhanced Three-Dimensional Magnetic Resonance Angiographic Assessment of the Pulmonary Artery Anatomy in Cyanotic Congenital Heart Disease With Pulmonary Stenosis or Atresia: Comparison With Cineangiography

Pulmonary stenosis (PS) or pulmonary atresia (PA) is an important component of complex cyanotic congenital heart disease, especially in tetralogy of Fallot or lesions with ventricular septal defect (VSD)-PS physiology. Management strategy in these patients depends on accurate assessment of PAs and identification of all sources of pulmonary blood flow. X-ray cineangiography is the "gold standard" for this purpose, but it has the inherent risks of an invasive procedure. Gadolinium-enhanced three-dimensional magnetic resonance angiography (Gd-MRA) has been shown to noninvasively and accurately evaluate various lesions of the vascular system. This study was undertaken to evaluate the accuracy of Gd-MRA compared with cineangiography in the evaluation of pulmonary anatomy. Nineteen patients having complex cyanotic heart disease with PS or PA were included in the study. All patients underwent Gd-MRA and cineangiography. Catheterisation and MRA findings regarding the anatomic variable of interest were analysed for agreement by Bland-Altman analysis. There was total agreement between the two modalities in the delineation of confluent PAs. McGoon's ratio and the Nakata index, which are standard measures of the adequacy of PA size, also showed excellent agreement between the two modalities. MRA was able to delineate all aorto-pulmonary collaterals in the setting of PA. MRA can delineate all sources of pulmonary blood supply in cyanotic congenital heart disease with PS and/or PA as well as provide accurate assessment of PA size for planning corrective surgery.

Preoperative evaluation of pulmonary artery morphology and pulmonary circulation in neonates with pulmonary atresia--usefulness of MR angiography in clinical routine.

BackgroundTo explore the role of contrast-enhanced magnetic resonance angiography (CE-MRA) in clinical routine for evaluating neonates with pulmonary atresia (PA) and to describe their pulmonary artery morphology and blood supply.CE-MRA studies of 15 neonates with PA (12 female; median weight: 2900 g) were retrospectively evaluated by two radiologists in consensus. Each study was judged to be either diagnostic or non-diagnostic depending on the potential to evaluate pulmonary artery morphology and pulmonary blood supply. In those cases where surgery or conventional angiocardiography was performed results were compared.ResultsCE-MRA was considered diagnostic in 87%. Pulmonary artery morphology was classified as "confluent with (n = 1) and without (n = 1) main pulmonary artery", "non-confluent" (n = 6) or "absent" (n = 7). Source of pulmonary blood supply was "a persistent arterial duct" (n = 12), "a direct" (n = 22) or "indirect (n = 9) aortopulmonary collateral artery (APCA)" or "an APCA from the ascending aorta" (n = 2). In no patient were there any additional findings at surgery or conventional angiocardiography which would have changed the therapeutic or surgical approach.ConclusionsCE-MRA is a useful diagnostic tool for the preoperative evaluation of the morphology of pulmonary arteries and blood supply in neonates with PA. In most cases diagnostic cardiac catheterization can be avoided.

Role of 64-MDCT in Evaluation of Pulmonary Atresia With Ventricular Septal Defect

The purposes of this article are to describe the technical factors in performing 64-MDCT of pulmonary atresia with ventricular septal defect (PA-VSD), to describe the anatomic variations in pulmonary arterial circulation and other associated anomalies, and to describe the postoperative evaluation of this condition. The management of this complex condition has been largely determined by the morphology of pulmonary arteries and the extracardiac sources of pulmonary blood supply. Although echocardiography and catheter angiography are the traditional imaging techniques used to diagnose PA-VSD, 64-MDCT has become a valuable noninvasive imaging technique in comprehensive evaluation of this condition.

Stenting of the arterial duct in newborns with duct-dependent pulmonary circulation

Objective:To evaluate the feasibility and results of stenting of the arterial duct in newborns with duct-dependent pulmonary circulation using low-profile, high-flexibility premounted coronary stents.Design:Prospective interventional and clinical follow-up study.Setting:Tertiary referral...

Novel application of 4D sonography with B‐flow imaging and spatio‐temporal image correlation (STIC) in the assessment of the anatomy of pulmonary arteries in fetuses with pulmonary atresia and ventricular septal defect

To assess the reliability of two-dimensional gray-scale (2D) and color Doppler echocardiography in the study of the size and anatomy of the central pulmonary arteries and of the sources of pulmonary blood flow in a case series of fetuses with pulmonary atresia and ventricular septal defect (PA-VSD), and to evaluate whether the use of 4D ultrasound with B-flow imaging and spatio-temporal image correlation (STIC) can improve prenatal diagnostic accuracy. The study population comprised a group of seven PA-VSD fetuses that had been examined by 2D and color Doppler echocardiography exclusively, and a group of five additional cases identified initially by conventional echocardiography and examined further by 4D ultrasound, for all of which a thorough postnatal or autopsy study of the size and anatomy of the pulmonary arteries and blood supply was available. 2D and color Doppler echocardiography failed to assess the anatomy of the central pulmonary arteries and the source of the pulmonary blood supply in 33% and 25% of the 12 cases, respectively. 4D ultrasound with B-flow imaging and STIC assessed successfully the anatomy of the pulmonary arteries and the source of pulmonary blood supply in all five fetuses examined. The prognosis of PA-VSD is influenced by the anatomy of the pulmonary arteries and the sources of the pulmonary blood supply, and by coexisting extracardiac and genetic anomalies. Our findings, although limited to a small sample size, suggest that 4D echocardiography with B-flow imaging and STIC, unlike 2D ultrasound, can provide thorough visualization of very small vessels and of the arterial blood supply to the lungs of fetuses with PA-VSD. 4D ultrasound may be used in the future to improve and help to detail the diagnosis of other fetal cardiac defects.

Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA) with ventricular septal defect (VSD). A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an ejection systolic murmur at the pulmonary area. ECG demonstrated sinus rhythm with normal axis and biventricular hypertrophy. Echocardiography was performed and truncus arteriosus (TA) type I was suspected with perimembranus VSD, overriding of the aorta, and dilated main pulmonary artery. But on cardiac catheterization studies, a non obstructive fistula was found between the left coronary and main pulmonary artery coexisted with PA and VSD. A successful surgery was performed subsequently and confirmed the above diagnosis. Although there were episodes of pulmonary hypertension crisis during early post operative course, she was then discharge from the hospital in a good condition. Since irreversible pulmonary vascular disease may develop in a non restrictive coronary to pulmonary fistula, early recognition of this anomaly is very important for better surgical result. (Med J Indones 2004; 13: 237-40)

Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease.

A clinical study on the outcomes of Glenn anastomoses performed since 1987 in eight consecutive patients aged > or = 16 years, and in two performed earlier, showed poor results. One badly selected patient died early as a consequence of high venous pressure, while a further seven had early complications. Seven of eight hospital survivors were followed for 1-10 (median 4.2) years with two deaths (1 and 4 years later). Of the remaining five patients, two improved temporarily, but increased arterial oxygen saturation was not maintained after 6 months. The two patients who had undergone a Glenn anastomosis 10 and 34 years earlier were shown to have pulmonary arteriovenous fistulas. The Glenn anastomosis in these older patients is associated with high rates of complication and appears not to give adequate palliation, particularly when it is the only source of pulmonary blood supply. In the adult, the Glenn anastomosis can be used as a staging procedure for Fontan-type surgery, but must be combined with another source of pulmonary arterial supply. Any adult having a Glenn anastomosis, particularly without another source of pulmonary arterial supply, should be warned of the possibility of worsening of cyanosis and symptoms. The second stage of the procedure may need to be performed soon after the first should the hypoxia prove intolerable.

Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect

The purpose of this study was to determine the role of high resolution two-dimensional echocardiographic imaging and color flow Doppler study in assessing the pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD). Although echocardiography is a well established technique for assessing central pulmonary arteries in pulmonary atresia VSD and for determining the presence or absence of a patent arterial duct, few data are available on its role in patients whose source of blood supply is from collateral vessels. Forty-two patients aged a few hours to 19 months (mean 29 days) were prospectively assessed by high resolution echocardiography, including color flow Doppler study, during a 4-year period ending in 1994, before any intervention other than intravenous administration of prostaglandins. Angiographic confirmation was available in 29 patients, including 18 (95%) of 19 with aortopulmonary collateral channels. A patent arterial duct was correctly identified as the sole source of pulmonary blood supply in 23 patients, whereas aortopulmonary collateral channels were detected in 19, with one of these having a small patent arterial duct and collateral channels. The patent arterial duct originated from the undersurface of the aorta in 16 (67%) of 24 patients and from the base of the brachiocephalic trunk in 7 (33%) of 24. All patients with a patent ductus as the sole source of pulmonary blood supply had confluent pulmonary arteries. Nonconfluent pulmonary arteries were present in six patients, with all but one having aortopulmonary collateral channels as the sole source of pulmonary flow. Aortopulmonary collateral channels were direct in 17 (89%) of 19 patients, whereas in 2 (11%) of 19, both direct and indirect collateral channels were present. Color flow Doppler study was accurate in determining the presence or absence, the side and the origin of the collateral channels in all patients, with the correct number being determined in 12 (67%) of 18. "Wash-in" to the hilar pulmonary arteries (retrograde color flow) was seen in 12 (92%) of 13 patients with collateral channels and confluent pulmonary arteries. Failure to identify a tiny central pulmonary artery occurred in one patient. High resolution imaging and color flow Doppler study provide good appreciation of the source of pulmonary blood supply in neonates and young infants with pulmonary atresia VSD.

Fistulous communications between a solitary coronary artery and the pulmonary arteries as the primary source of pulmonary blood supply in tetralogy of fallot with pulmonary valve atresia

A communication between the coronary and pulmonary arteries, described previously only in isolated cases 1–3 and a series of 5 patients in which the communication was called an “aortopulmonary tunnel,” 4 is a rare source of pulmonary arterial supply in tetralogy of Fallot with pulmonary atresia. Between 1954 and 1987, we encountered 4 patients with tetralogy of Fallot and pulmonary atresia in whom the pulmonary flow to one or both lungs was via a fistulous communication between a coronary artery and the intrapericardial pulmonary arteries. They form the basis of this report.

Solitary arterial trunk with pulmonary atresia and arteries with supply to the left lung from both an arterial duct and systemic-pulmonary collateral arteries

We report a case of pulmonary atresia with ventricular septal defect and absent pulmonary trunk. The sources of pulmonary blood supply were the arterial duct and systemic pulmonary collateral arteries. The left lung received blood from both sources which is very rare. One of the systemic pulmonary collateral arteries arose from the right coronary artery in an unusual fashion.

Bronchial compression by a patent ductus arteriosus associated with pulmonary atresia

Two infants with pulmonary atresia are presented, wherein a large, vertically oriented patent ductus arteriosus, acting as the only source of pulmonary blood supply, compressed the left bronchus, causing significant respiratory distress. Hyperinflation of the left lung was the clue to this circumstance. After bronchographic confirmation, surgical repair was accomplished by placement of aortopulmonary shunts and division of the patent ductus. Other causes of airway compression in congenital heart disease are briefly reviewed. The embryology of the ductus arteriosus is discussed, emphasizing its anatomic configuration and physiologic role in pulmonary atresia.

Duplicate sources of pulmonary blood supply in pulmonary atresia with ventricular septal defect.

In pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, there is a tendency for each collateral artery to be the sole supply to a particular region of lung. On injection into a collateral artery, however, "wash-out" of contrast medium by non-opacified blood from a second source is sometimes seen. Alternatively, contrast medium may faintly "wash-in" to an adjacent lobar artery supplied from a second source. "Wash-in" and "wash-out" therefore show that a duplicate blood supply exists. To assess the importance of these phenomena, we reviewed the angiograms of 50 patients with this condition. Duplicate pulmonary blood supply not resulting from surgical shunts was found in 25 patients (50%), and in 24 of 37 patients (65%) who had selective collateral injections. In one patient two collaterals anastomosed with each other. Thirty-nine instances of duplicate supply occurred in the remaining 24. The duplicate connection was to a region of lung connected to a central pulmonary artery in 29 of 39 instances (74%). Determination of how much of the peripheral pulmonary vasculature is connected to central pulmonary arteries greatly affects decisions about palliative and corrective surgery. These results show that unless "wash-in" and "wash-out" are specifically looked for, the information obtained from angiography may be erroneous.