Nonneoplastic diseases of aorta and pulmonary trunk—Part V

This five-part review focuses on selected nonneoplastic diseases of the aorta and pulmonary trunk. Because many more diseases affect the aorta compared with the pulmonary trunk and the right and left main pulmonary arteries, most of this review will be devoted to disorders of the aorta. Part V of this five-part series on diseases of the aorta and the pulmonary trunk focuses on nonneoplastic diseases of the pulmonary trunk and the right and left main pulmonary arteries.     

Nonneoplastic diseases of aorta and pulmonary trunk—Part II

This five-part review focuses on selected nonneoplastic diseases of the aorta and pulmonary trunk. Because many more diseases affect the aorta compared with the pulmonary trunk and right and left main pulmonary arteries, most of this review will be devoted to disorders of the aorta. Part II of this five-part series discusses complications of aortic dissection and aortic aneurysm.     

Nonneoplastic diseases of aorta and pulmonary trunk—Part iv

This five-part review focuses on selected nonneoplastic diseases of the aorta and pulmonary trunk. Because many more diseases affect the aorta compared with the pulmonary trunk and right and left main pulmonary arteries, most of this review will be devoted to disorders of the aorta. Part IV of the series discusses cystic medial degeneration, trauma, and congenital diseases of the aorta.     

Utility of three-dimensional volume rendering images using electron-beam computed tomography to evaluate possible causes of ischemia from an anomalous origin of the right coronary artery from the left sinus of valsalva.

The present study evaluated the usefulness of 3-dimensional volume rendering (VR) images using electron-beam computed tomography (EBCT) in determining the possible causes of ischemia resulting from the anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva, which coursed between the ascending aorta and pulmonary trunk. Such anomalies could cause ischemia or sudden death without obstructive coronary artery disease. The suggested mechanism is either compression causing closure of the slit-like orifice of the anomalous artery as the aorta dilates with exertion or compression of the anomalous artery by the aorta and pulmonary trunk as it courses between these 2 arteries, which dilate with exercise. A 17-year-old male underwent EBCT coupled with a 100-ml intravenous injection of iodinated contrast medium. Data were reconstructed into 3-dimensional images through VR to evaluate the shape of the orifice and the spatial relationship of the RCA, ascending aorta and pulmonary trunk. Perspective VR showed the shape of the orifice of the left main trunk, which was not slit-like, and cut-plane VR showed the spatial relationship of both the lumen and the surface of the RCA, ascending aorta and pulmonary trunk, providing information on whether the ascending aorta or pulmonary trunk would compress the RCA and cause ischemia.     

Cell lineages and tissue boundaries in cardiac arterial and venous poles: developmental patterns, animal models, and implications for congenital vascular diseases

Multiple cell populations with different embryological histories are involved in the morphogenesis of the cardiac arterial and venous poles as well as in the correct alignment and connection of the developing vessels with the cardiac chambers. Formation of the aorta and the pulmonary trunk is a complicated process orchestrated via a specific sequence of highly integrated spatiotemporal events of cell proliferation, migration, differentiation, and apoptosis. The peculiar susceptibility of this intricate cell network to be altered explains the frequency of congenital cardiovascular diseases of the arterial and venous poles. We review this topic from the "vascular point of view," putting major emphasis on (1) the existence of different cell lineages from which smooth muscle cells of the aorticopulmonary trunk can be derived, (2) the establishment of cell/tissue boundaries in the cardiovascular connecting regions, and (3) the animal models that can mimic human congenital defects of the arterial and venous poles of the heart.     

Ortner's syndrome, presentation of two cases with cardiovocal hoarseness

We present two cases of Ortner's syndrome, a syndrome with hoarseness caused by unilateral left recurrent laryngeal nerve palsy as a complication of cardiovascular diseases. In the first case, aneurysmatic dilatation of the aorta and in the second case a dilated pulmonary trunk was thought to be the cause.     

Anomalous origin of the left main coronary artery from the right sinus of Valsalva

The clinical and angiographic findings in 10 patients with anomalous origin of the left main (LM) coronary artery from the right sinus of Valsalva are presented. The initial course of the anomalous artery was between the aorta and the pulmonary trunk in 6 patients (5 men and 1 woman), and posterior to the aorta in 4 patients (3 women and 1 man). The best coronary angiographic projection to determine the course of the LM coronary artery in relation to the aorta and pulmonary trunk was the right anterior oblique and lateral projections, with a catheter in the pulmonary trunk as a guide. In the right anterior oblique projection left ventriculogram, an increased density at the base of the aorta was seen in all patients with posterior-to-aorta course of the anomalous artery. The blood supply to the first portion of the ventricular septum is provided by 1 or 2 descending septal branches from the anomalous LM artery, when the anomalous vessel is coursing between the aorta and pulmonary trunk. When the anomalous LM courses posteriorly to the aorta, it does not provide any septal branches. In patients with this anomaly, the blood supply to the first portion of the septum is usually provided by descending septal branches originating from the right sinus of Valsalva or from the first portion of the right coronary artery. Six of the 10 patients had severe obstructive coronary artery disease. Four of the 6 patients had significant LM coronary artery disease.     

Banding of the pulmonary trunk in preparation for a Fontan operation.

Banding of the pulmonary trunk is an important surgical procedure for patients who have congenital cardiac malformations with unrestricted pulmonary flow. We propose a new concept for determining in such circumstances the most appropriate length of the band used to constrict the pulmonary trunk in preparation for a Fontan operation. We studied 14 patients undergoing banding of the pulmonary trunk and measured the following parameters: diameter of aorta, diameter of pulmonary trunk, length of pulmonary arterial band and maximum flow velocity across the banded segment. We calculated an index from our original parameter based on the formula; length of band/(diameter of aorta diameter of pulmonary trunk). The diameter of aorta was 9.5 +/- 1.4 mm, and that of the pulmonary trunk was 9.6 +/- 2.3 mm. The length of the band was 16.5 +/- 3.4 mm, giving a calculated index of 0.188 +/- 0.038. The maximum flow velocity was 4.02 +/- 0.46 m/s. No correlation was found between the length of the band and body weight, and also no correlation was found between the length of the band and maximum flow velocity. The calculated index had a negative correlation with the maximum velocity of flow across the band (y = -8.13x +/- 5.56, R = 0.74, p < 0.01). We believe that the proposed index is a useful guide in determining the length of a pulmonary band when preparing patients for a Fontan operation.     

冠状动脉畸形及其临床意义

目的 :探讨冠状动脉造影的人群中冠状动脉畸形的检出率、临床特点和预后。　　方法 :回顾性总结 3 188例行冠状动脉造影术的人群。　　结果 :3 188例行冠状动脉造影术的人群中 ,共检出冠状动脉畸形 42例 ,检出率为 1 3 2 % ,其中 81%为冠状动脉起源和分布异常 ,19%为冠状动脉静脉瘘 ;76 2 %为对心肌灌注没有影响、相对良性的畸形 ,2 3 8%为对心肌灌注有潜在影响、有一定的危险性的畸形。　　结论 :右冠状动脉起源于左冠状动脉窦、左主干起源于右冠状动脉窦并穿行于主动脉和肺动脉之间、左主干起自肺动脉等是非常严重的冠状动脉畸形 ,患者常发生心绞痛、急性心肌梗死和心力衰竭 ,这些畸形一旦发现 ,应进行预防性的手术矫正。小冠状动脉瘘多数无临床症状 ,但多发和大冠状动脉瘘可发生充血性心力衰竭、心肌缺血。冠状动脉畸形并非罕见 ,某些类型的冠状动脉畸形可致严重的临床后果 ,应尽早进行预防性地手术矫正     

Importance and potentials of angiocardiographic study in the diagnosis of congenital heart defects]

Contemporary requirements to angiocardiographic examination in congenital heart diseases are discussed. It is shown that patients with high pulmonary hypertension should be examined by means of left ventriculography and aortography which permit the diagnosis of intraventricular septal defect combined with patent ductus arteriosus, common arterial trunk and open atrioventricular canal. Auxiliary methods of examination in such a commonly encountered anomaly as Fallot's tetralogy and atresia of the pulmonary artery are described, which make it possible to judge the condition of the pulmonary artery. The necessity is demonstrated for performing a two- and multiserial examination in complete and corrected transposition of the major vessels, dual origin of the aorta and pulmonary artery from the right ventricle, and in cases with a solitary ventricle. The role of angiocardiography in appraising the volume of cardiac cavities in patients with congenital heart diseases is noted.     

Abnormal origin of the right pulmonary artery from the ascending aorta. Echocardiographic diagnosis apropos of 2 cases

Anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital malformation. Formerly identified at angiography and during surgery, it can now be diagnosed by two-dimensional echocardiography, as was done in the two cases reported here. The first case was a neonate in heart failure in whom echocardiography revealed the anomalous origin of the right pulmonary artery; this diagnosis was confirmed at post-mortem examination. The second case was a 4-month old infant in heart failure; echocardiography displayed the malformation which was confirmed at angiography and surgery. In both cases echocardiography showed discontinuity between the pulmonary trunk and the right pulmonary artery and continuity between the pulmonary trunk and the left pulmonary artery. There also was continuity between the right pulmonary artery and the ascending aorta. Doppler ultrasound showed a systolic and a diastolic flow in the right pulmonary artery and only a systolic flow in the left pulmonary artery. A colour-coded doppler examination in one case confirmed the anomalous origin of the right pulmonary artery and the absence of any other abnormality. Two-dimensional echocardiography combined with colour-coded doppler ultrasound therefore is the method of choice to diagnose anomalous origin of the right pulmonary artery from the ascending aorta.     

Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.     

Transposition of the great arteries with posterior aorta and subaortic conus: anatomical and surgical correlation

Three hearts studied at autopsy with transposition of the great arteries and posterior aorta with isolated or predominantly subaortic conus are described. In all cases the aorta was posterior and to the right of the pulmonary trunk. In two cases it was significantly distant from the pulmonary trunk and in one it was very close. The caliber of the pulmonary trunk was larger than that of the aorta: > 2:1 in two cases and < 2:1 in one case. A well developed subaortic conus was seen in all cases and a small subpulmonary conus in one case. In the other two cases, the pulmonary valve was in fibrous continuity with the mitral valve. In all cases the aortic valve was higher than the pulmonary valve, the relative heights above the ventricles being $\text{25}\text{20}$, $\text{25}\text{30}$ and $\text{40}\text{60}\text{mm}$, respectively. The left coronary artery ran posteriorly and to the left of the pulmonary trunk in all the cases. The length of the main stem of the left coronary artery was 11, 11 and 30 mm, respectively. We discuss the significance of this type of transposition of the great arteries for techniques currently used for surgical correction.     

Ascending aortic and pulmonary trunk aneurysms

A case of two non-atherosclerotic aneurysms localised in the ascending aorta and in the pulmonary trunk is presented. Histopathologically, a severe granulomatous inflammation affecting the whole aneurysms wall was documented. To the best of our knowledge it is the second ever documented case of simultaneous occurrence of aneurysms in the aorta and the pulmonary artery.     

Persistent truncus arteriosus: A study of 66 autopsy cases with special reference to definition and morphogenesis

Sixty-six hearts were examined in which a single arterial trunk, leaving the base of the heart through a single semilunar valve, supplied the aorta, pulmonary artery and coronary arteries. Careful attention was paid to the infundibular morphologic features in these hearts, and these were compared with findings in 24 hearts with single aortic trunk, pulmonary atresia and ventricular septal defect. It was concluded that the two anomalies represented morphologically discrete conditions. This was particularly true with regard to the disposition of the infundibular septum, the ventriculo-infundibular fold and the relation of the coronary arteries to the semilunar sinuses. Although it is theoretically possible for a heart with true persistent truncus arteriosus to have absence of the pulmonary trunk and right and left pulmonary arteries, it is argued that such hearts (“truncus type IV”) are best classified as pulmonary atresia with ventricular septal defect. It is also argued that hearts with a common arterial trunk supplied through discrete ventricular outflow tracts and two semilunar valves are best considered examples of aorticopulmonary window. It is suggested that persistent truncus arteriosus is best defined as that condition in which a single arterial trunk leaves the heart through a single semilunar valve and supplies the aorta, one or both pulmonary arteries and the coronary arteries.     

Anomalous origin of one pulmonary artery from the ascending aorta

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.     

Three-dimensional reconstruction of computed tomographic images of anomalous origin of the left main coronary artery from the pulmonary trunk in an adult

We present a 49-year-old female case of anomalous origin of the left main coronary artery from the pulmonary trunk. Multidetector computed tomography was performed, and 3-dimensional reconstruction of computed tomographic images found that the left main coronary artery originated from left sinus of the pulmonary trunk and the right coronary artery from the right coronary cusp of the aorta. We speculate that this patient's long life may be due to the dominant right coronary artery and rich collateral from the right coronary artery to the left coronary artery.     

Isolated chylopericardium after intrapericardial procedures: possible role of inadvertent right efferent lymphatic trunk injury

Chylopericardium after an intrapericardial procedure is rare, and satisfactory explanations of its possible causes are lacking.Herein, we present 4 cases of chylopericardium that developed after intrapericardial surgery, and we review the literature.Our literature review revealed 29 cases of chylopericardium that complicated intrapericardial operations, to which we added our 4 cases for analysis. The 33 surgical procedures involved repair for congenital heart disease (n=21), valve surgery (n=5), coronary artery bypass grafting (n=6), and other (n=1). Causes were verified in 7 patients: small lymphatic injury in 3 and high venous pressure or venous thrombosis in 4. Of the 26 patients with chylopericardium of unknown origin, 15 had congenital heart disease. Ten of these 15 had chromosomal abnormalities, especially trisomy 21 (Down syndrome); these patients typically had increased lymphatic permeability, which raised the likelihood of chylopericardium. Five revascularizations for coronary artery disease required harvesting of the left internal thoracic artery for reconstruction, incurring a risk of damage to the drainage site of the right efferent lymphatic trunk. In addition, all 26 patients with chylopericardium of unknown origin underwent dissection of the ascending aorta and the main pulmonary artery, near the right efferent lymphatic trunk. Inadvertent injury to the trunk during the dissection would have increased the risk of chylopericardium. Accordingly, even though the overall incidence of chylopericardium during intrapericardial procedures is low, we recommend a meticulous dissection of the ascending aorta from the main pulmonary artery.     

Anomalous origin and course of the coronary arteries

In the normal heart, the right and left coronary arteries arise from the aortic valvar sinuses adjacent to the pulmonary trunk. The right coronary artery then directly enters the right atrioventricular groove, whereas the main stem of the left coronary artery runs a short course before dividing to become the anterior interventricular and circumflex arteries. These arteries can have an anomalous origin from either the aorta or pulmonary trunk; their branches can have various anomalous origins relative to arterial pedicles. Other abnormal situations include myocardial bridging, abnormal communications, solitary coronary arteries, and duplicated arteries. Understanding of these variations is key to determining those anomalous patterns associated with sudden cardiac death. In the most common variant of an anomalous origin from the pulmonary trunk, the main stem of the left coronary artery arises from the sinus of the pulmonary trunk adjacent to the anticipated left coronary arterial aortic sinus. The artery can, however, arise from a pulmonary artery, or the right coronary artery can have an anomalous pulmonary origin. The key feature in the anomalous aortic origin is the potential for squeezing of the artery, produced by either the so-called intramural origin from the aorta, or the passage of the abnormal artery between the aortic root and the subpulmonary infundibulum.     

The frontal chest film as a method of suspecting transposition complexes.

The frontal chest film as a means of suspecting transposition complexes is discussed. The first step is recognizing the normal relationships formed by the ascending aorta, an aortic knob-descending aorta, and pulmonary trunk. The concept of which ventricle is connected to which atrium is developed--the terms ventricular noninversion and inversion being utilized. Frontal chest film signs of transposition are summarized as follows: 1) in the majority of transposition complexes, absence of the pulmonary trunk is the premier sign; 2) patients with ventricular noninversion tend to show the ascending aorta and aortic knob-descending aorta in normal position. Patients with ventricular inversion usually show absence of all three great artery relationships. 3) In ventricular inversion, the left heart border shows a septal notch or a diffuse convex bulge (two ventricles), or a discrete bulge high up on the left border (single ventricle).