A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis

Congenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our center for further evaluation. We carried out an angiography which revealed the absence of the right pulmonary artery and multiple collaterals originating from the right subclavian and right internal mammary arteries supplying the right lung. During the follow-up the patient developed a severe episode of pulmonary infection and pulmonary hypertension which responded well to medical treatment. Physicians should be aware of the congenital absence of the right pulmonary artery especially in patients presenting with recurrent respiratory symptoms. Although this condition is generally considered to have a good prognosis, close observation is mandatory in order to prevent further complications and comorbidities.     

Absent pulmonary artery

Summary Cardiac catheterization was performed in nine patients with unilateral absence of a pulmonary artery. Aortography revealed a diverticulum of the innominate artery in five of six patients in whom the aortic arch and absent pulmonary artery were on opposite sides. It is suggested that the diverticulum indicates fetal systemic blood supply to the affected lung through the distal part of the sixth aortic arch, which—consisting of ductal tissue—obliterated after birth, leaving the diverticulum where the ductus joins the innominate artery.     

Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced,reversible, left-sided pulmonary hypertension

Summary An infant with scimitar syndrome, absent right pulmonary artery, and systemic blood supply to the right lung presented in severe cardiac failure. Cardiac catheterization revealed suprasystemic pressure of the left pulmonary artery and a high pulmonary vascular resistance. Right-sided pneumonectomy abolished cardiac failure and normalized both pulmonary artery pressure and resistance. Pure volume load affecting one lung—as in this case through absence of the right pulmonary artery plus additional left-to-right shunt from a systemic collateral—can lead to pulmonary hypertension. Early operative intervention can reverse this process and prevent pulmonary vascular disease.     

Congenital unilateral pulmonary venous atresia with pulmonary veno-occlusive disease in contralateral lung: An unusual association

Summary A patient presenting with the rare association of congenital unilateral pulmonary venous atresia in one lung and pulmonary veno-occlusive disease in the other is described. The patient first presented at the age of 3 &frac; years with anemia, hemoptysis, and pulmonary hypertension. After cardiac catheterization and angiocardiography, a diagnosis of atresia of the left pulmonary veins was made for which left pneumonectomy was done. Four years later the patient presented with right-sided congestive failure and radiologic evidence of right-sided pulmonary edema from which death resulted. At autopsy, the right lung showed changes of pulmonary veno-occlusive disease, while the major veins were not involved.     

Isolated Unilateral Agenesis of the Pulmonary Artery: Surgical Repair With an Artificial Graft

This report describes a 3-month-old Japanese boy with a diagnosis of isolated unilateral agenesis of the proximal right pulmonary artery with severe pulmonary hypertension. One-stage reconstruction of the right pulmonary artery was performed without cardiopulmonary bypass. The hilar right pulmonary artery and the distal main pulmonary artery were joined by anastomosis to an artificial ring graft. The boy's postoperative course was uneventful, and the pressure in the pulmonary artery was within the normal range. Although mild right pulmonary artery stenosis remained, the authors' therapeutic strategy may provide a clinically important option for isolated unilateral agenesis of the pulmonary artery.     

Unilateral pulmonary edema and acute rheumatic fever

Although the diagnostic criteria for acute rheumatic fever (ARF) are well known, a high index of suspicion is necessary in order to assure timely diagnosis and appropriate treatment. We present a case of an 8-year-old child who presented with unilateral pulmonary edema secondary to acute mitral insufficiency due to ARF. ARF should be considered in the differential diagnosis of unilateral pulmonary edema in children.     

Imperforate tricuspid valve with dysplasia of the right ventricular myocardium,pulmonary valve,and coronary artery: A clinicopathological study of nine cases

Summary Nine cases of imperforate tricuspid valve associated with dysplasia of the right ventricular myocardium, pulmonary valve, and right coronary artery are described. The mean frontal QRS axis of the electrocardiograms did not indicate left axis deviation in seven of the nine. Two-dimensional echocardiograms showed an imperforate tricuspid valve, normally aligned atrioventricular septum, and an irregular-shaped right ventricular cavity with a thin wall. In three cases with absence of the pulmonary valve, pulsed Doppler echocardiograms of the right ventricular outflow tract revealed antegrade flow during systole and retrograde flow during diastole, and selective angiography through a persistent ductus arteriosus showed retrograde filling of the blind-ended right ventricular cavity via the main pulmonary artery. Necropsy examinations showed an imperforate tricuspid valve with a thin-walled aneurysmal right ventricle in all six cases examined. Pulmonary atresia was observed in two cases, and the absence of the pulmonary valve in four. The right coronary artery was hypoplastic in four cases and absent in two. A shunt operation should be carried out in early infancy to prevent hypoxia after closure of the ductus arteriosus.     

Clinical Manifestations and Long-Term Follow-Up in Pediatric Patients Living at Altitude With Isolated Pulmonary Artery of Ductal Origin

This study's aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.     

Asthma-like Attacks Resulting from the Isolated Congenital Left Pulmonary Artery Agenesis with Right Main Bronchus Stenosis

A 5-year-old girl with isolated congenital left pulmonary artery agenesis suffered from recurrent attacks of dyspnea and right-sided pneumonia due to the stenosis of the right main bronchus. The division of ligamentum of the ductus arteriosus and suspension of the right pulmonary artery resulted in the disappearance of symptoms. It is notable that the compression of contralateral bronchus by the remaining pulmonary artery can cause respiratory symptoms in patients with isolated unilateral pulmonary artery agenesis.     

Takayasu's arteritis presenting as pulmonary hypertension

Takayasu's arteritis (pulseless disease) is a vasculitis that affects the large arteries, including the aorta, its branches, and the pulmonary arteries. A 15-year-old girl with Takayasu's arteritis was referred to our institution for marked pulmonary hypertension in the absence of symptoms referable to systemic vasculitis. She was shown to have a thickened, narrowed thoracic aorta, a stenosed right axillary artery, and severely affected pulmonary arteries. Despite corticosteroid therapy she died one month after diagnosis. This patient's illness indicates that pulmonary vessels might be the principal vessels involved in Takayasu's arteritis.     

The significance of lung scintigraphy in anomalies of the pulmonary vascular bed

Aplasia, hypoplasia and stenosis of the pulmonary artery showed, on scintigraphic investigation, unilateral, homogenous decreased in lung perfusion. In 3 of 4 children with pulmonary sequestration there was a difference in blood flow between the two lungs, or in one part of the lung. Isolated anomalous drainage of pulmonary veins does not produce deviations from the normal distribution of blood flowing from the right ventricle into the lungs. Analysis of scinitigraphic findings in 1450 children with cardiopulmonary disease has shown that with an otherwise unexplained marked homogeneous unilateral decrease in flow, we can with great certainty diagnose a developmental defect of the pulmonary vessels. Differences in lung area, judged from the scintigram, are a sensitive indicator of associated disturbances of development of the lung parenchyma. In 26 of 42 such developmental anomalies of the pulmonary vascular bed this finding was demonstrated. Pulmonary scintigraphy is a very valuable complementary method, because of its simplicity and relative safety for the patient, in the diagnosis of congenital anomalies of the pulmonary vasculature, especially in infants and young children.     

Causes of congenital unilateral pulmonary hypoplasia: a study of 33 cases

A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of the ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia.     

Tetralogy of Fallot with Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta and Hypoplastic Left Pulmonary Artery

A case of tetralogy of Fallot associated with anomalous origin of the right pulmonary artery from the ascending aorta and hypoplastic left pulmonary artery was successfully repaired. Following the simultaneous procedure with banding of the right pulmonary artery and replacement of modified Blalock-Taussig shunt to the left pulmonary artery at 34 days of age, the intracardiac repair was performed successfully at 1 year and 10 months of age. The patient was well 6 months postoperatively, without unilateral pulmonary hypertension.     

Fetal hypertension and the development of increased pulmonary vascular smooth muscle: a possible mechanism for persistent pulmonary hypertension of the newborn infant.

Chronic pulmonary arterial hypertension was produced in six fetal lambs. In four (126 to 139 days' gestation) unilateral fetal renal artery constriction caused systemic arterial mean blood pressure elevations. In another fetus, constriction of the umbilical artery caused a systemic mean blood pressure elevation; in the sixth, partial occlusion of the ductus arteriosus caused isolated pulmonary arterial hypertension. The right lung of each fetus was perfused with fixative at the in vivo mean arterial pressure and the amount of smooth muscle in the fifth generation (resistance) vessels analyzed using the medial width/external diameter ratio. There was a significant increase in the medial width/external diameter ratio in the six experimental animals as compared to that in six normal fetuses. In separate fetuses the increased ratios were due to a decreased external diameter, increased smooth muscle, or both these factors. The total number of resistance vessels was counted in the right lung of each fetus and no significant difference from normal was observed. We postulate that either fetal systemic hypertension or constriction of the ductus arteriosus causes fetal pulmonary hypertension in utero and that this produces increased smooth muscle development in pulmonary arterial resistance vessels; this may be a pathogenic mechanism for the syndrome of persistent pulmonary hypertension of the newborn infant.     

Tricuspid atresia, hypoplastic right ventricle, intact ventricular septum and congenital absence of the pulmonary valve.

A case of tricuspid atresia by sealing of the valves, associated with an intact ventricular septum, a hypoplastic right ventricle and the absence of the pulmonary valve, is described. The foramen ovale and the ductus arteriosus were widely patent but there was no interventricular communication. The pulmonary flow was assured by the ductus which had a saccular continuation in the pulmonary trunk. It is postulated that the lesions probably occurred during the period between the XIX and XXII horizons and that the malformed right coronary artery may have played an important role in producing the lesions of the right ventricle.     

肺静脉指数对肺血减少型先天性心脏病肺血管发育的评估

目的 探索能更准确反映肺血管发育及肺血流情况的指标,为外科手术方案的选择提供依据.方法 采用74例肺血减少型先天性心脏病心血管造影序列,测量左右肺动脉及4根肺静脉直径,分别计算Nakata指数,McGoon指数,肺静脉指数(PVI),分别与术后情况进行相关分析.结果 左、右侧肺静脉大小分别与左右肺动脉大小高度相关,左侧肺静脉与左肺动脉远端的相关性为0.73,左侧肺静脉与左肺动脉近端的相关性为0.72,右侧肺静脉与右肺动脉远端的相关性为0.67,右侧肺静脉与右肺动脉近端的相关性为0.71.PVI与术后监护时间,呼吸机维持时间,正性肌力药物用量的相关性分别为-0.51,-0.478和-0.693,均比Nakata指数,McGoon指数明显增高,能更准确的反映整个肺血管的发育情况.右室流出道重建术后无低心排组与低心排组的McGoon指数分别为1.97±0.58与1.36±0.51(t=2.347,P＜0.05),两组Nakata指数分别为(269±124)mm2/m2和(164±106)mm2/m2(t=2.218,P＜0.05),PVI分别为(273±125)mm2/m2和(152±77)mm2/m2(t=2.936,P＜0.01),低心排组肺血管值均明显小于无低心排组.当PVI小于180 mm2/m2时,术后易出现血流动力学不稳定,低心排,甚至死亡.结论 肺动脉、肺静脉发育彼此相关,PVI能更准确反映肺血管发育及肺血流情况的形态学指标,为外科手术方案的选择提供有效依据.     

Unilateral negative-pressure pulmonary edema in an infant during bronchoscopy

We report here a case of a 3-week-old infant with a lung disease of unknown origin who underwent diagnostic fiber-optic bronchoscopy. During the procedure, she developed unilateral pulmonary edema. Factors that contributed to this complication are discussed along with means to reduce its occurrence.     

Coexistent transient pulmonary edema and pericardial effusion

Eight (23%) of 35 children with acute pericardial effusions due to infection or juvenile rheumatoid arthritis (JRA) had associated transient pulmonary edema demonstrated on plain chest radiographs. The presence or absence of radiographic pulmonary edema correlated well with clinical and hemodynamic parameters in patients with JRA but not in those with infectious pericarditis. There was no definite relationship between radiographic edema and amount of pericardial fluid as estimated echocardiographically or removed at pericardiocentesis. Rapidity of pericardial fluid accumulation could not be assessed in this study. Children of young age with underlying JRA were the most likely subjects to have radiographic pulmonary edema in conjunction with an acute pericardial effusion.Presented in part at the 28th Meeting of The Society of Pediatric Radiology, Boston 1985     

Percutaneous pulmonary valve replacement

Each year in the United States more than 4000 newborns are diagnosed with complex congenital heart disease involving right ventricular outflow tract obstruction. Despite advances in surgical techniques with right ventricle to pulmonary artery conduits, progressive conduit failure often occurs within 4–12 years. Consequently, the majority of these patients, prior to becoming adults, require 2–4 operations for the right ventricle outflow tract to reverse the adverse ventricular effects of a volume or pressure load. Fortunately, over the past decade, percutaneous pulmonary valve replacement has emerged as a nonsurgical intervention for right ventricle to pulmonary artery conduit failure.Currently, there are two percutaneous pulmonary valves available in the United States with the Edwards SAPIEN? valve actively enrolling in a Phase II FDA approved clinical trial, and the Medtronic Melody® valve now FDA approved through a Humanitarian Device Exception pathway. In this article, we review indications, technique and outcomes for percutaneous pulmonary valve replacement and compare and contrast the available percutaneous stent valves.