A case of isolated tricuspid valve infective endocarditis caused by Abiotrophia defectiva

One patient with major aorto-pulmonary collateral arteries and heterotaxy underwent staged unifocalizations, and eventually the staged Fontan completion. Subsequent to the bidirectional Glenn procedure, the left pulmonary artery was once thrombozed, but successfully treated. The progressively regurgitant common atrioventricular valve needed repair twice during the course. Pulmonary arterial pressure was 11 mm Hg 1 year after the Fontan procedure.     

Catheter closure of accessory pulmonary blood flow after bidirectional Glenn anastomosis using Amplatzer duct occluder.

Additional source of pulmonary blood flow in patients with bidirectional cavopulmonary anastomosis (Glenn shunt) may cause elevation of the pulmonary artery pressure precluding safe completion of the Fontan operation. A case is presented with single-ventricle bidirectional cavopulmonary anastomosis and additional flow from the ventricle to the pulmonary artery resulting in elevated Glenn pressure. The communication was successfully occluded using Amplatzer duct occluder with satisfactory reduction in the Glenn pressure.     

New formula for predicting mean pulmonary artery pressure using systolic pulmonary artery pressure

STUDY OBJECTIVES: Mean pulmonary artery pressure (MPAP) and systolic pulmonary artery pressure (SPAP) are used interchangeably to define pulmonary hypertension (PH). We tested the hypothesis that the measurement of MPAP and SPAP is redundant in resting humans over a wide pressure range. DESIGN: Prospective, observational study. SETTING: Catheterization laboratory in a university hospital. PATIENTS: This study involved 31 patients, as follows: primary PH, nine patients; chronic pulmonary thromboembolism, seven patients; venous PH, six patients; and control subjects with normal pulmonary artery pressure, nine patients. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: High-fidelity pulmonary artery pressures were obtained when patients were at rest. Over the wide MPAP range that was under study (10 to 78 mm Hg), MPAP and SPAP were strongly related (r(2) = 0.98). Regression analysis performed on the first 16 subjects (test sample) allowed us to propose a formula (MPAP = 0.61 SPAP + 2 mm Hg), the accuracy of which was confirmed in the remaining 15 subjects (validation sample bias, 0 +/- 2 mm Hg). If PH was defined by an SPAP in excess of 30 or 40 mm Hg, this corresponded to an MPAP in excess of 20 or 26 mm Hg. If PH was defined by an MPAP of > 25 mm Hg, this corresponded to an SPAP of > 38 mm Hg. CONCLUSIONS: In resting humans, MPAP can be accurately predicted from SPAP over a wide pressure range. The new formula may help to refine the threshold pressure values used in the diagnosis of PH. Further studies are needed to test the hypothesis that our formula may allow the noninvasive prediction of MPAP from Doppler-derived SPAP values.     

2岁以上合并重度肺动脉高压先天性心脏病行单心室修复的初步研究

目的：探讨2岁以上合并重度肺动脉高压先天性心脏病（先心病）行单心室修复的手术指征及效果。方法：回顾性分析年龄〉2岁合并重度肺动脉高压仅能行单心室修复的先心病患儿15例,男性9例,女性6例。年龄2.0～10岁,平均（7.8±3.8）岁。体质量11～24kg,平均（15.32±4.24）kg,术前经皮血氧饱和度（SPO2）88%～95%,平均（91.75±2.29）%。肺动脉压（mPAP）52～91mmHg（（1 mm-Hg=0.133 kPa））,平均（54.61±16.8）mmHg。心脏畸形：单心室7例,三尖瓣闭锁3例,室缺远离两大动脉伴左心室发育不良型右心室双出口2例,心室不均衡型完全型房室通道2例,十字交叉心1例。均在静吸复合麻醉下先期行肺动脉环缩术,后期逐步完成双向格林或全腔肺动脉吻合术。结果：全组无住院死亡;PAB术前后mPAP、SPO2明显下降;PAB术后3～6年内完成双向格林（Glenn）4例,全腔肺动脉吻合术（TCPC）2例。结论：PAB术能有效降低肺动脉压力,≥2岁PAB术后可进一步完成Glenn或TCPC术。     

Outcome of single ventricle and total anomalous pulmonary venous connection

Management of patients with single-ventricle physiology is significantly affected by anomalies of pulmonary venous return at all stages, whether primary palliation, bidirectional Glenn shunt, or completion of Fontan circulation. We treated 25 patients with pulmonary venous anomalies and single ventricle by staged palliation, from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 of them. Primary palliation with a systemic-to-pulmonary artery shunt was undertaken in 15 patients. There were 5 early deaths, of which 4 were due to obstruction of pulmonary venous return. A bidirectional Glenn shunt was constructed in 17 patients including 10 who had it as a primary palliative procedure. There were 7 early deaths after the bidirectional Glenn procedure; only one was due to pulmonary venous obstruction. Five patients attained completion of the Fontan procedure. There was one early death after the Fontan operation. Anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle, with an impact on survival in early infancy. Palliation with the aim of instituting extracardiac conduit Fontan circulation allows greater latitude and more streamlined management.     

Hemiazygos Venous Additional Pulmonary Flow for Successful Total Cavo‐pulmonary Connection

Patients who underwent only Glenn procedure after being deemed unsuitable candidates for Fontan completion are not small in number, and may develop arterio‐venous (AV) pulmonary malformations during the follow‐up period. We present the case of a 17‐year‐old woman with severe systemic desaturation 13 years after Glenn procedure and repair of total anomalous pulmonary venous return. Among other anomalies, the patient disclosed hemiazygos continuation to a persistent left superior vena cava. This case supports the concept that returning flow from the abdominal vein plays an important role in the well‐balanced growth of the pulmonary artery and in the inhibition of the pulmonary AV malformation.     

Occlusion of Sano conduit with the Amplatzer Vascular Plug: A reliable method for staged elimination of accessory pulmonary blood flow in single ventricle palliation

Background : Staged palliative surgery is often required to regulate pulmonary and systemic blood flow in children with complex congenital heart disease. Accessory sources of pulmonary blood flow have been utilized to provide satisfactory pulmonary blood flow after Glenn anastomosis in select patients. Prolonged exposure to increased flow can lead to elevated pulmonary arterial pressures and volume loading of the ventricle, potentially limiting candidacy for Fontan completion. Objective : To review the feasibility, technique, and outcome of transcatheter closure of the Sano conduit utilizing the Amplatzer Vascular Plug (AVP). Methods : All patients who underwent device closure of the Sano conduit with an AVP were reviewed retrospectively. Patient demographics, indications, procedural details, and patient outcomes were reviewed. Hemodynamic changes before and after occlusion of the Sano conduit were compared. A P value < 0.05 was considered significant. Results : Between 2005 and 2009, 11 male patients underwent Sano occlusion with an AVP. Average weight was 8.8 ± 2.2 kg. The Sano was occluded from both antegrade (n = 6) and retrograde (n = 5) approaches. Arterial saturation and mean pulmonary artery pressure both decreased significantly after Sano occlusion [86.9 ± 4.9% to 82.6 ± 4.9% (P < 0.001) and 14.1 ± 5 mm Hg to 12.8 ± 4 mm Hg (P < 0.05), respectively]. Conduit diameter averaged 3.6 mm (range 3.1–4.4) 13 months after Stage I palliation. The conduit was closed using a 6 mm AVP in nine patients and a 4 mm AVP in the other two. The ratio of AVP size to conduit diameter was 1.5 ± 0.27. There were no procedural complications and all patients at least 2 years of age have undergone Fontan completion. Conclusions : The Sano conduit can be left open in select patients who do not tolerate closure after Glenn anastomosis. It can be closed safely and effectively using the AVP. © 2010 Wiley‐Liss, Inc.     

A flow restrictor implanted percutaneously across a loose pulmonary artery band

Pulmonary artery banding usually is performed as a palliative procedure. In patients with elevated pulmonary vascular resistance and complex congenital heart disease, it may not be possible at the time of surgery to obtain sufficient restriction to optimize the patient for further treatment; additional restriction may be needed in time. We present a technique where we used a combination of two devices (a 10‐mm Amplatzer ASD occluder fenestrated with a Palmaz Genesis 9/19 mm stent) to percutaneously further reduce the flow to the lungs 1 month after surgical placement of a pulmonary artery band in a 16‐year‐old girl with complex univentricular heart. Eight months after the banding the patient successfully underwent completion of the Fontan circulation by total cavopulmonary connection. This technique allows to reduce flow without redo surgery. In well‐selected patients, this new percutaneous technique allows to better prepare such complex patients for future surgery. © 2011 Wiley‐Liss, Inc.     

Aortic origin of the right pulmonary artery associated with ductus arteriosus in an adult. A case report

We describe the case of a 26-year-old female in functional class I (NYHA), with aortic origin of the right pulmonary artery associated with a persistent ductus arteriosus and severe pulmonary artery hypertension (101/40-70 mm Hg), which remained elevated (89/40-60 mm Hg) after the administration of 100% oxygen. Right pulmonary artery pressure (125/60-86 mm Hg) was higher than that of main pulmonary artery and similar to aorta pressure. The patient was successfully treated: surgical closure of the ductus arteriosus and end-to-end anastomosis between the pulmonary artery and right pulmonary artery were carried out. Systolic pulmonary arterial pressure, estimated by echocardiography Doppler, was 60 mm Hg six months after surgery. Cross-sectional echocardiogram showed the anastomosis of the right pulmonary artery with the main pulmonary artery. Pulmonary gammagraphy showed both lungs perfused through the main pulmonary artery; right lung perfusion was lesser than left lung perfusion, 30 vs. 70% respectively. Aortic origin of a right or left pulmonary artery is a heart disease seen in patients during the course of the first year of life. Its frequency is < 1% among all the congenital cardiopathies and the survival rate to adult life is very low. The originality of this paper is the presentation of a rare congenital cardiopathy treated surgically in an adult.     

The pulmonary artery closure may be a thromboembolic risk factor in patients with bidirectional cavo-pulmonary (Glenn) shunt

Postoperative thrombosis after the Fontan procedure has been well noted in the literature, and its risk factors are also well known. In contrast, thrombosis after the bilateral cavo-pulmonary shunt (Glenn) has been rarely reported and almost always occurs around the anastomosis itself or near it, mainly causing pulmonary embolism. We present 2 cases with cerebral embolism 2-7 months after pulmonary artery closure and Glenn procedure, due to dislodgement of a thrombus in the proximal pulmonary artery stump. Based on these two cases and a few others reported in the literature, we want to call the attention to this new cause of thromboembolism after Glenn and stimulate discussion about its incidence, risk factors and preventive measures.     

Diurnal variation of pulmonary artery pressure in chronic heart failure

Variation in pulmonary artery pressure has important consequences for the interpretation of isolated pressure measurements in patients with chronic heart failure. To investigate the nature of diurnal variation in pulmonary artery pressure in chronic heart failure, eight angina-free men (aged 50-72 years) with treated chronic heart failure caused by ischaemic heart disease underwent continuous ambulatory pulmonary artery pressure recording by a transducer tipped catheter. The mean (1 SD) daytime pulmonary artery pressure was 29.6 (5.0) mm Hg systolic and 13.7 (5.6) mm Hg diastolic. The mean change in pressure from day to night was +5.1 (3.2) mm Hg systolic and +3.8 (1.7) mm Hg diastolic; and the mean change from standing to lying +9.3 (2.3) mm Hg systolic and +6.4 (2.1) mm Hg diastolic. In six of the eight patients there was considerable rise in pulmonary artery pressure at night, but in the two patients with the most severe symptoms there was no nocturnal rise. In patients with chronic heart failure, nocturnal pulmonary artery pressure is not determined by postural change alone. But interpretation of isolated pulmonary artery pressure measurements must take the posture of the patient into account.     

Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk

Ventricular dysfunction, elevated pulmonary vascular resistance, and residual distal pulmonary artery distortion contribute to early mortality after a Fontan operation; they may be transient or reversible. A baffle fenestration, allowing right-to-left shunting, maintains cardiac output and limits right atrial pressure. A baffle fenestration was surgically created at the time of a modified Fontan repair in 20 consecutive patients. Risk factors included pulmonary artery pressure of 18 mm Hg or more, end-diastolic pressure of 12 mm Hg or more, valvar regurgitation, pulmonary artery distortion, pulmonary vascular resistance of 2 Woods' units or more, ventricular outflow obstruction, and complex anatomy. Nineteen of 20 patients survived. After the operation, mean arterial oxygen saturation was 86%, mean right atrial pressure was 15 mm Hg, and mean duration of pleural effusions was 6 days. Twelve of 19 survivors tolerated early test occlusion and had permanent transcatheter umbrella closure. Four patients failed early test occlusion, with a significant decrease in venous O2 saturation and a rise in central venous pressure, due to ventricular dysfunction, pulmonary artery distortion, or aortopulmonary collaterals. Three of four had successful late closure of the fenestration after correction of these abnormalities.     

Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

BACKGROUND. We reviewed the outcome of 76 consecutive patients (age range, 5 months to 6 years; median age, 19 months) who underwent a modified Fontan procedure after initial palliative surgery for hypoplastic left heart syndrome (HLHS) between January 1984 and December 1989. METHODS AND RESULTS. Modifications of the Fontan procedure included transatrial baffle of pulmonary venous return to the tricuspid valve (n = 10) or inferior vena cava baffle within the right atrium to the superior vena caval-pulmonary artery anastomosis, with pulmonary artery augmentation (n = 66). Actuarial survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52% (4 years). Of the 43 survivors, 25 patients have returned for postoperative cardiac catheterization at a medium of 13 months after the Fontan procedure. Mean +/- SD hemodynamic values were cardiac index, 2.8 +/- 0.6 l/min/m2; right arterial pressure, 11 +/- 2 mm Hg; pulmonary artery wedge pressure, 6 +/- 3 mm Hg; and arterial oxygen saturation, 94 +/- 3%. No patient had significant tricuspid or native pulmonary valve insufficiency. CONCLUSIONS. Survival after the Fontan procedure in patients with HLHS is comparable to survival after a Fontan procedure in patients with other complex congenital heart lesions. In the subgroup of patients with HLHS who survived both reconstructive surgery and a Fontan procedure and have been evaluated by cardiac catheterization after a Fontan procedure, the use of the right ventricle as the systemic ventricle yielded excellent intermediate results for Fontan physiology.     

Diurnal variation of pulmonary artery pressure in chronic heart failure.

Variation in pulmonary artery pressure has important consequences for the interpretation of isolated pressure measurements in patients with chronic heart failure. To investigate the nature of diurnal variation in pulmonary artery pressure in chronic heart failure, eight angina-free men (aged 50-72 years) with treated chronic heart failure caused by ischaemic heart disease underwent continuous ambulatory pulmonary artery pressure recording by a transducer tipped catheter. The mean (1 SD) daytime pulmonary artery pressure was 29.6 (5.0) mm Hg systolic and 13.7 (5.6) mm Hg diastolic. The mean change in pressure from day to night was +5.1 (3.2) mm Hg systolic and +3.8 (1.7) mm Hg diastolic; and the mean change from standing to lying +9.3 (2.3) mm Hg systolic and +6.4 (2.1) mm Hg diastolic. In six of the eight patients there was considerable rise in pulmonary artery pressure at night, but in the two patients with the most severe symptoms there was no nocturnal rise. In patients with chronic heart failure, nocturnal pulmonary artery pressure is not determined by postural change alone. But interpretation of isolated pulmonary artery pressure measurements must take the posture of the patient into account.     

Percutaneous use of stents to correct pulmonary artery stenosis in young children after cavopulmonary anastomosis

Pulmonary artery distortion is a risk factor among candidates for the Fontan procedure. In 57 patients evaluated by catheterization after successful cavopulmonary anastomosis, 8 had proximal left pulmonary artery (LPA) stenosis, either discrete (4 patients) or long segment (4 patients). Median age was 27 months (range 19 to 60 months). Median weight was 11.4 kg (range 9.1 to 20.0). Median diameter at LPA stenosis was 4.4 ± 0.4. Proximal right pulmonary artery mean diameter was 10.4 ± 1.0 mm. After angiographic and hemodynamic assessment, short 11F sheaths were placed in the right internal jugular (6 patients) or subclavian veins (2 patients). Pulmonary artery angioplasty and stent placement were performed. LPA stenoses were enlarged using 10 Palmaz stents dilated to 10 mm (7 patients) or to 12 mm (3 patients). Poststent angiograms showed that narrowest LPA dimensions were significantly enlarged to 9.9 mm ± 1.0 mm, p < 001). There were no complications. Follow-up studies (catheterizations in 4 patients, echocardiograms in 8 patients) were performed 4 to 9 months after stent implantation. No restenosis was observed. Five patients had completion of their Fontan procedures; three patients are pending Fontan completion. This study demonstrates the efficacy and safety of the percutaneous use of Palmaz stents to correct pulmonary artery stenosis in young children after cavopulmonary anastomosis.     

Single-stage versus two-stage modified Fontan procedure

We compared surgical outcomes of the single-stage and two-stage modified Fontan procedures to clarify clinical superiority. Of 28 children undergoing a modified Fontan procedure from October 1995 to October 2005, 15 had a 1-stage and 13 had a 2-stage operation. In the 2-stage group, pulmonary artery growth was evaluated before and after the first stage. Operative mortality was 26.6% in the 1-stage group and 0% in the 2-stage group. The benefits of a previous bidirectional Glenn shunt were decreased cyanosis and volume overload, but there was no significant difference in pulmonary artery growth reflected in pulmonary artery indices before and after the bidirectional Glenn procedure. Older children underwent a 2-stage modified Fontan procedure and had better outcomes in terms of lower mortality, improved oxygen saturation, decreased volume load, and less deterioration of atrioventricular valve regurgitation.     

Pulmonary artery pulse pressure and wave reflection in chronic pulmonary thromboembolism and primary pulmonary hypertension

OBJECTIVES: The purpose of this time-domain study was to compare pulmonary artery (PA) pulse pressure and wave reflection in chronic pulmonary thromboembolism (CPTE) and primary pulmonary hypertension (PPH). BACKGROUND: Pulmonary artery pressure waveform analysis provides a simple and accurate estimation of right ventricular afterload in the time-domain. Chronic pulmonary thromboembolism and PPH are both responsible for severe pulmonary hypertension. Chronic pulmonary thromboembolism and PPH predominantly involve proximal and distal arteries, respectively, and may lead to differences in PA pressure waveform. METHODS: High-fidelity PA pressure was recorded in 14 patients (7 men/7 women, 46 +/- 14 years) with CPTE (n = 7) and PPH (n = 7). We measured thermodilution cardiac output, mean PA pressure (MPAP), PA pulse pressure (PAPP = systolic - diastolic PAP) and normalized PAPP (nPAPP = PPAP/MPAP). Wave reflection was quantified by measuring Ti, that is, the time between pressure upstroke and the systolic inflection point (Pi), deltaP, that is, the systolic PAP minus Pi difference, and the augmentation index (deltaP/PPAP). RESULTS: At baseline, CPTE and PPH had similar cardiac index (2.4 +/- 0.4 vs. 2.5 +/- 0.5 l/min/m2), mean PAP (59 +/- 9 vs. 59 +/- 10 mm Hg), PPAP (57 +/- 13 vs. 53 +/- 13 mm Hg) and nPPAP (0.97 +/- 0.16 vs. 0.89 +/- 0.13). Chronic pulmonary thromboembolism had shorter Ti (90 +/- 17 vs. 126 +/- 16 ms, p < 0.01) and higher deltaP/PPAP (0.26 +/- 0.01 vs. 0.09 +/- 0.07, p < 0.01). CONCLUSIONS: Our study indicated that: 1) CPTE and PPH with severe pulmonary hypertension had similar PA pulse pressure, and 2) wave reflection is elevated in both groups, and CPTE had increased and anticipated wave reflection as compared with PPH, thus suggesting differences in the pulsatile component of right ventricular afterload.     

Dissecting aneurysm of the pulmonary artery with pulmonary hypertension

Pulmonary artery dissection was observed in a 64-yr-old female patient with severe pulmonary hypertension, which was probably primary (pulmonary vascular resistance, 817 dyn.s.cm-5; normal range less than or equal to 200 dyn.s.cm-5). The patient was admitted to the hospital because of severe dyspnea on exertion. Echocardiography demonstrated a dissecting aneurysm of the pulmonary artery. Right heart catheterization revealed severe pulmonary hypertension (mean pulmonary artery pressure, 64 mm Hg; normal range, 10 to 22 mm Hg); dissection of the pulmonary artery was confirmed by pulmonary arteriography. One-year follow-up was uneventful. In the literature, 28 patients with dissecting aneurysm of the pulmonary artery are reviewed. The dissection has only been diagnosed in life in one patient (by echocardiography).     

Percutaneous transluminal balloon dilatation of the mitral valve in pregnancy

Pregnancy can cause life threatening complications in women with mitral stenosis, and there is a substantial risk of fetal death if valvotomy under cardiopulmonary bypass is required. A patient is described in whom pulmonary oedema developed after delivery of her first child by caesarean section 13 months previously. Subsequent cardiac catheterisation showed severe mitral stenosis (valve area 0.96 cm2, valve gradient 12 mm Hg, pulmonary artery pressure 30/16 mm Hg). Before valvotomy could be performed the patient again became pregnant and presented in pulmonary oedema at twenty two weeks' gestation. Medical treatment was unsuccessful and she underwent percutaneous transluminal balloon dilatation of the mitral valve. This increased the valve area to 1.78 cm2 and reduced the transmitral gradient to 6 mm Hg. The procedure was uncomplicated, and she remained symptom free on no medication. She delivered vaginally at 37 weeks' gestation. Percutaneous transluminal balloon dilatation of the mitral valve is a safe and effective alternative to mitral valvotomy in pregnancy.     

犬肺血栓栓塞后不同时间的血气、血液动力学及血栓的病理变化

目的建立犬肺血栓栓塞症(PTE)模型;观察栓塞后不同时间犬动脉血气、血液动力学、影像学及血栓病理的变化情况。方法健康成年杂种犬16只,其中栓塞左下肺动脉的15只按随机数字表法随机分为3组,每组5只:假手术组;1周组:5段血栓柱栓塞,观察1周;2周组:5段血栓柱栓塞,观察2周。另外1只将血栓柱栓塞至右下肺动脉以证实选择性栓塞的可行性,观察2周。观察指标为动脉血气、血液动力学参数及局部肺动脉造影影像学变化。实验犬经血栓栓塞制模后肌注氨甲环酸,1、2周后解剖观察栓塞情况;应用磷钨酸苏木精染色(PTAH)观察血栓的病理变化。结果犬肺血栓栓塞前,氧合指数(PaO_2/FiO_2)为(508±58)mm Hg(1 mm Hg=0.133 kPa),栓塞1 h 后为(395±100)mm Hg;栓塞前平均肺动脉压(MPAP)为(15±3)mm Hg,栓塞1 h后为(21±4)mm Hg;肺血管阻力(PVR)栓塞前为(178±114)mm Hg·s/L,栓塞1 h后为(404±260)mm Hg·s/L,差异均有统计学意义(均 P<0.05)。实验犬肺动脉栓塞后局部肺动脉造影可见截断征;1周时见栓塞近端肺动脉明显增粗,管壁僵硬样改变等。栓塞1、2周时解剖发现实验犬肺动脉内血栓表面不平滑;PTAH 染色见血栓表面机化,2周组见血栓内多处再通,肺动脉壁增生组织包绕、分割血栓。结论将血栓柱栓塞犬肺叶动脉,并用氨甲环酸抑制纤溶,可建立模拟慢性 PTE 部分病理改变的动物模型;栓塞不同时间,肺动脉造影表现不同;时间越长,血栓机化越明显。