右后外侧小切口剖胸治疗冠状动脉右心室和/或右心房瘘

目的:总结经右外侧腋下小切口入胸体外循环辅助直视下治疗冠状动脉右心室右心房瘘的手术经验及效果。方法:选取本院2002年1月至2018年12月,行冠状动脉右心室和/或右心房瘘矫治术患儿31例,其中经胸骨正中开胸完成手术矫治的患儿16例(正中组),男性9例,女性7例,中位年龄11.8个月(6～32)个月,中位体质量12.7 kg(7.5～29 kg)。其中右冠状动脉右心室瘘9例,左冠状动脉右心室瘘5例,右冠状动脉右心房瘘2例;经右外侧小切口剖胸(右侧组)行手术矫治的患儿15例,男性9例,女性6例。中位年龄10.8个月(5～28个月),中位体质量11.6 kg(5.6～18)kg。右冠状动脉右心室瘘9例,左冠状动脉右心室瘘4例,右冠状动脉右心房瘘2例。均在全身麻醉体外循环辅助下完成冠状动脉右心室和/或右心房瘘矫治术。结果:右侧组术后无死亡。术后残余瘘2例。正中死亡1例,死于低心排出量综合征(低心排);术后残余瘘2例。右侧组手术时间、切口长度、ICU停留时间、术后引流量、术后输血量、术后呼吸机辅助时间以及住院时间都显著低于正中组;主动脉阻断时间差异无统计学意义。随访3～38个月,心脏功能恢复良好。结论:经右侧腋下小切口直视下行冠状动脉右心室右心房瘘矫治手术,手术安全可靠,术野清晰,创伤小,术后恢复良好。     

Usefulness of echocardiographic right ventricular measurements in estimating right ventricular hypertrophy and right ventricular systolic pressure

Right ventricular wall thickness (T) measurements were made in 111 patients by echocardiography to evaluate their usefulness in diagnossing right ventricular hypertrophy (RVH) and in predicting right ventricular peak systolic pressure (P). Anatomic and echocardiographic findings of RVH were compared in 36 of 111 subjects: diastolic T (dT) and systolic T (sT) had a sensitivity of 90 and 34% and a specificity of 94 and 100%, respectively, in the diagnosis of RVH. Echocardiographic and hemodynamic findings were compared in the remaining 75 of 111 patients undergoing cardiac catheterization: dt was 6.5 +/- 2.7 mm in 46 patients with elevated P (58.2 +/- 30.2 mm Hg) versus 3.8 +/- 0.9 in 29 patients with normal P (26.3 +/- 2.7 mm Hg) (p less than 0.01). The dT and P had a linear correlation (r = 0.92) in 40 patients with right ventricular end-diastolic dimension less than 13 mm/m2. Estimates of P in 22 patients with atrial septal defect and right ventricular end-diastolic dimension greater than 13 mm/m2 were fairly good (r = 0.83). It is concluded that echocardiographic measurements of T are useful in diagnosing RVH and in estimating P.     

Isolated right ventricular aneurysm following right ventricular infarction

A 78-year-old man with atherosclerotic heart disease developed extensive right ventricular infarction fibrosis with aneurysm formation following right coronary artery occlusion. No symptoms of right-sided heart failure were present. Postmortem examination revealed that 40% of the right ventricle, 11% of the septum and 7% of the left ventricular free wall were infarcted due to right coronary artery occlusion. This is the first documented case of isolated aneurysm of the right ventricle following infarction and it demonstrates that even extensive right ventricular destruction may be present without symptoms.     

Rupture of right coronary artery aneurysm into the right atrium

A 63-year-old man presented with acute congestive heart failure and was found to have a continuous murmur. Two years earlier, he had an inferior myocardial infarct, when no murmurs were heard. Angiography showed a right coronary artery aneurysm communicating with the right atrium. The distal vessel was occluded. The aneurysm was resected and the patient remains well. It is proposed that this was a congenital aneurysm which led firstly to the myocardial infarct and finally ruptured into the right atrium.     

Detection of acute right ventricular infarction by right precordial electrocardiography

The value of 0.1 mV or greater of S-T segment elevation in at least one right precordial lead (V₄R to V₆R) in defining right ventricular myocardial infarction was assessed prospectively in 43 subjects (33 consecutive patients with enzymatically confirmed infarction of varying type and location, 4 patients with unstable angina and 6 healthy volunteers). Patients with acute myocardial infarction were studied with radionuclide ventriculography and technetium-99m stannous pyrophosphate myocardial scintigraphy 18.2 ± 14.3 (mean ± standard deviation) and 85.1 ± 18.0 hours after the onset of symptoms, respectively. Eleven patients (Group A: 9 patients with transmural inferior infarction, 1 with transmural inferolateral infarction and 1 with transmural anteroseptal infarction) demonstrated right precordial S-T segment elevation and 22 patients (Group B: 6 patients with transmural inferior infarction, 2 with transmural posterior infarction, 3 with transmural inferolateral infarction, 3 with transmural anteroseptal infarction, 3 with transmural extensive anterior infarction, 4 with subendocardial anterior infarction and 1 with unclassified infarction) did not. Right ventricular ejection fraction was significantly lower in Group A (0.47 ± 0.11) than in Group B (0.60 ± 0.12) (p < 0.01). Right ventricular total wall motion score was 63.8 ± 15.6 percent of normal in Group A versus 94.3 ± 8.5 percent in Group B (p < 0.001). Technetium-99m pyrophosphate uptake (2+ or greater) over the right ventricle occurred in nine patients (81.8 percent) in Group A and in one patient (4.5 percent) in Group B (p < 0.001). No patient with unstable angina and no healthy volunteer had S-T segment elevation in a right precordial lead. S-T segment elevation of 0.1 mV or greater in one or more of leads V₄R to V₆R is both highly sensitive (90 percent) and specific (91 percent) in identifying acute right ventricular infarction.     

Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy

Diagnostic sensitivity and specificity of cineangiography were evaluated by multivariate logistic discriminant analysis in 32 patients with arrhythmogenic right ventricular (RV) cardiomyopathy, 27 patients with biventricular dilated cardiomyopathy, 28 patients with atrial septal defect and 18 normal subjects. In patients with arrhythmogenic RV cardiomyopathy and biventricular dilated cardiomyopathy, the diagnosis was confirmed by endomyocardial biopsy. All RV values overlapped for the diagnosis of atrial septal defect and arrhythmogenic RV cardiomyopathy; overlapping extended to dilated cardiomyopathy for end-diastolic volume and infundibular dimensions. RV ejection fraction appeared reduced in all the diseases; in particular, mean values in dilated cardiomyopathy and arrhythmogenic RV cardiomyopathy were 38 and 53%, respectively (p less than 0.05). Left ventricular quantitative studies showed a significant difference between dilated and arrhythmogenic RV cardiomyopathy, both in terms of pumping indexes (mean end-diastolic volumes 180 vs 91 ml/m2 and mean ejection fraction 33 vs 60%), and indexes of contractility (stress/end-diastolic volume 3.7 vs 6.7). Multivariate analysis disclosed that transversally arranged hypertrophic trabeculae, separated by deep fissures, were associated with the highest probability of arrhythmogenic RV cardiomyopathy (p less than 0.001). Posterior subtricuspid and anterior infundibular wall bulgings were the only other independently significant variables. Coexistence of these signs was associated with 96% specificity and 87.5% sensitivity. Thus, arrhythmogenic RV cardiomyopathy presents quantitative volumetric and hemodynamic as well as qualitative features that clearly distinguish it from dilated cardiomyopathy and confirm its nosographic autonomy among the primary diseases of the myocardium.     

Initial report of percutaneous right ventricular assist for right ventricular shock secondary to right ventricular infarction.

A 57-year-old female suffered an acute inferior ST segment elevation myocardial infarction. The patient failed thrombolysis and was urgently transferred for rescue percutaneous coronary intervention of the right coronary artery. She decompensated after reperfusion of the occluded RCA and developed cardiogenic shock from severe right heart failure refractory to IABP support and maximal pressors. A percutaneous right ventricular assist device was successfully implanted, which improved mean arterial pressure to a viable range and allowed withdrawal of inotropic medications.Right ventricular failure after infarction remains difficult to manage and has a high mortality. Intraaortic balloon pump and LVAD support have not proven beneficial in cardiogenic shock secondary to RV infarction. This is a report of the first insertion of a percutaneous right ventricular assist device for right ventricular support in a human. Further evaluation is warranted to evaluate the potential benefits of such a device as well as optimal timing of initiation of RV support.     

Transcatheter closure of right coronary artery fistula to the right ventricle

Coronary artery fistula (CAF) is an uncommon anomaly that is usually congenital but can be acquired. Although most patients are asymptomatic, some may present with congestive heart failure, infective endocarditis, myocardial ischemia or rupture. In the past, surgical ligation was the only option in the management of CAF, but since 1983, transcatheter closure of CAF has been increasing as an alternative to surgery. We report a 3-year-old boy, presented in Queen Alia Heart Institute, who underwent successful transcatheter closure of a large fistula communicating the distal part of the right coronary artery to the right ventricle. Our case differs from other CAFs in that the fistula was communicating the right coronary artery itself to the right ventricle.