We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot＇s...We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot＇s tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.展开更多
The left pulmonary artery sling （LPAS） is a rare vascular anomaly causing respiratory distress in which theleft pulmonary artery arises from the posterior aspect of the right pulmonary artery, courses posteriorly to...The left pulmonary artery sling （LPAS） is a rare vascular anomaly causing respiratory distress in which theleft pulmonary artery arises from the posterior aspect of the right pulmonary artery, courses posteriorly to the right of the bronchus and passes between the trachea and oesophagus to reach the hilum of the left lung. The LPAS is frequently associated with tracheobronchial tree anomalies and congenital cardiac defects. Proper assessment of the tracheobronchial and cardiovascular anomaly is essential in LPAS for planning management of the patient. Currently,展开更多
A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardi...A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.展开更多
Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary emb...Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.展开更多
Background：Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate t...Background：Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography. Methods： From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up. Results： The mortality rate after the operation was 11.5%. Before repair, twenty patients （76.9%） presented with left ventricular （LV） dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation （MR） was present in all patients. Two patients （7.7%）, both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months （range：0.5–36.0 months）. The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients.All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients （26.1%） showed supravalvar pulmonary stenosis and seven patients （30.4%） showed right pulmonary stenosis during follow-up. Conclusions： Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.展开更多
Background Anomalous Origin of Coronary Artery （AOCA）, a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It＇ s surgical treatment is scarcely performed in clinical practice...Background Anomalous Origin of Coronary Artery （AOCA）, a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It＇ s surgical treatment is scarcely performed in clinical practice, thus the special nursing needed during the surgery for this rare disease is largely unrecognized. Method There were 7 adult patients who had suffered from AOCA received corrective operation in our hospital in the last 10 years. Preoperative preparation, nursing cooperation and emergency procedure were analyzed and summarized. Result The AOCA corrective surgery was all successfully completed on these 7 patients. The nursing key points were drawn from these patients. Conclusion Key points of nursing cooperation in AOCA correction surgery include： understanding the condition, mastering pathological anatomy preoperative, making strict application of gradient temperature control ,observing the change of the disease, and carrying out all kinds of emergency preparation during surgery. Meanwhile, careful check in all cases and literature data, participate in preoperative discussion, and skilled techniques are also important to the success of surgery.展开更多
Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ...Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ventricle, together with mitral regurgitation. Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term. Method From May 1998 to July 2012, 38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction （33 received left coronary artery re-implantation, 4 left coronary artery ligation or primary closure, 1 Takeuchi procedure, and 10 simultaneous mitral valve plasty）. Left ventricular dimension, mitral regurgitation, and ejection fraction, were measured by color Doppler echocardiography preoperatively, and 1 month after discharge. Results Hospital survival was 94.7% （2 in-hospital deaths）. Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty, one of whom was converted to mechanical prosthetic valve replacement. Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction, 3 of whom also received additional mitral leaflet cleft repair. Meanwhile 8 patients underwent other different concomitant operations. Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic, endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mr, （P 〈 0.01） and 23.04 ±5.87 mm （P 〈 0.01） respectively. The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93 （P 〈 0.05） in the group. All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ±0.54 （P 〈 0.05）. Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective, and can get satisfactory dimensional and functional improvement of the left ventricle in early term. Although controversial, concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.展开更多
文摘We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot＇s tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.
文摘The left pulmonary artery sling （LPAS） is a rare vascular anomaly causing respiratory distress in which theleft pulmonary artery arises from the posterior aspect of the right pulmonary artery, courses posteriorly to the right of the bronchus and passes between the trachea and oesophagus to reach the hilum of the left lung. The LPAS is frequently associated with tracheobronchial tree anomalies and congenital cardiac defects. Proper assessment of the tracheobronchial and cardiovascular anomaly is essential in LPAS for planning management of the patient. Currently,
基金The project was funded by the Natural Science Foundation of Chongqing Science and Technology Commission(cstc2019jcyj-msxmX0866).
文摘A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.
文摘Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.
文摘Background：Anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography. Methods： From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up. Results： The mortality rate after the operation was 11.5%. Before repair, twenty patients （76.9%） presented with left ventricular （LV） dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation （MR） was present in all patients. Two patients （7.7%）, both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months （range：0.5–36.0 months）. The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients.All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients （26.1%） showed supravalvar pulmonary stenosis and seven patients （30.4%） showed right pulmonary stenosis during follow-up. Conclusions： Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.
基金supported by The National Five-Year Plan(No.2011BAI11B22)Guangdong province science and technology plan(No.2011A030400001)
文摘Background Anomalous Origin of Coronary Artery （AOCA）, a congenital heart disease with low incidence, can cause sudden cardiac death in the young. It＇ s surgical treatment is scarcely performed in clinical practice, thus the special nursing needed during the surgery for this rare disease is largely unrecognized. Method There were 7 adult patients who had suffered from AOCA received corrective operation in our hospital in the last 10 years. Preoperative preparation, nursing cooperation and emergency procedure were analyzed and summarized. Result The AOCA corrective surgery was all successfully completed on these 7 patients. The nursing key points were drawn from these patients. Conclusion Key points of nursing cooperation in AOCA correction surgery include： understanding the condition, mastering pathological anatomy preoperative, making strict application of gradient temperature control ,observing the change of the disease, and carrying out all kinds of emergency preparation during surgery. Meanwhile, careful check in all cases and literature data, participate in preoperative discussion, and skilled techniques are also important to the success of surgery.
基金supported by The Twelfth National Five-Year Plan (Grant No. 2011BAI11B22)
文摘Background Anomalous origin of the left coronary artery cardiac lesion resulting in myocardial ischemia even infarction, from the pulmonary artery is a rare congenital morphological impairment and dysfunction of left ventricle, together with mitral regurgitation. Here we will introduce our experience in the surgical repair of this kind of congenital lesion and the retrospective analysis about the improvement of left ventricular dimension and mitral regurgitation in early postoperative term. Method From May 1998 to July 2012, 38 consecutive patients with anomalous coronary artery from the pulmonary artery underwent surgical correction （33 received left coronary artery re-implantation, 4 left coronary artery ligation or primary closure, 1 Takeuchi procedure, and 10 simultaneous mitral valve plasty）. Left ventricular dimension, mitral regurgitation, and ejection fraction, were measured by color Doppler echocardiography preoperatively, and 1 month after discharge. Results Hospital survival was 94.7% （2 in-hospital deaths）. Ten paptients with more than moderate mitral regurgitation received simultaneous mitral plasty, one of whom was converted to mechanical prosthetic valve replacement. Mitral valve annuloplasty was applied in 9 cases of coronary re-implantation correction, 3 of whom also received additional mitral leaflet cleft repair. Meanwhile 8 patients underwent other different concomitant operations. Echocardiographic results for the survivals 1 month after discharge showed that left ventricular end-diastolic, endsystolic dimension decreased from 40.05 ± 5.56 mm and 28.94 ± 6.21 mm to 33.07 ± 6.82 mr, （P 〈 0.01） and 23.04 ±5.87 mm （P 〈 0.01） respectively. The average mitral regurgitation grade was also reduced from 2.36 ± 1.08 to 1.64 ± 93 （P 〈 0.05） in the group. All survival patients improved clinically and NYHA functional class decreased significantly from 2.37 ± 1.08 to 2.10 ±0.54 （P 〈 0.05）. Conclusions The surgical repair of anomalous origin of the left coronary artery from the pulmonary artery is safe and effective, and can get satisfactory dimensional and functional improvement of the left ventricle in early term. Although controversial, concomitant mitral valve plasty can be helpful for critical patients with severe mitral regurgitation.
