Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Sinus venosus atrial septal defect (SV‐ASD) usually coexists with partial anomalous pulmonary vein connection (PAPVC). It is a difficult diagnosis in transthoracic echocardiography (TTE) due to eccentric position of defects. We present a rare case of atypical anatomical variation in PAPVC, which was never described before. Two right pulmonary veins drained into superior vena cava, which overrode SV‐ASD and interatrial septum, a third pulmonary vein into the right atrium. Complete diagnosis could not be set after TTE, nor transesophageal echocardiography, whereas angio‐CT was finally conclusive. This diagnostic approach allowed the surgical planning.     

Pulmonary arterial hypertension associated with the use of interferon therapy for chronic hepatitis C infection complicated by extrinsic left main coronary artery compression

Interferon‐alpha treatment is a rare cause of pulmonary arterial hypertension (PAH). We report a case of a 43‐year‐old man treated for chronic hepatitis C infection complicated by decompensated right heart failure diagnosed with PAH and external coronary artery compression secondary to a dilated pulmonary trunk. The novel complication of extrinsic coronary artery compression should be considered in PAH patients presenting with chest pain or acute coronary syndrome. Establishing a diagnosis has clinical value as pulmonary vasodilator therapy may improve symptoms.     

Prenatal diagnosis of left pulmonary artery sling and review of literature

Left pulmonary artery sling (LPAS) is a very rare cause of large airway compression. In LPAS, the left pulmonary artery (LPA) arises from the proximal right pulmonary artery, coursing over the right mainstem bronchus, posterior to the trachea and anterior to the esophagus prior to reaching the left hilum. The aberrant course of the LPA results in anatomical obstruction of the right mainstem bronchus, the trachea, or both. Only a few reports present the prenatal features of LPAS. In this report, we present the prenatal diagnosis of a case of LPAS in one of a set of identical twins in which the only feature was that of an abnormal course of the LPA on 3‐vessel tracheal view. The cross‐sectional view at the level of three vessels which includes both pulmonary artery branches is useful to detect this abnormality. Color and power Doppler may be helpful as well.     

Two‐ and four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation in the diagnosis of fetal isolated partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three‐vessel and trachea view is useful for the diagnosis. Four‐dimensional echocardiography with high‐definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected.     

Acute pulmonary embolism: a concise review of diagnosis and management

An acute pulmonary embolism (aPE) is characterised by occlusion of one or more pulmonary arteries. Physiological disturbance may be minimal, but often cardiac output decreases as the right ventricle attempts to overcome increased afterload. Additionally, ventilation‐perfusion mismatches can develop in affected vascular beds, reducing systemic oxygenation. Incidence is reported at 50–75 per 100 000 in Australia and New Zealand, with 30‐day mortality rates ranging from 0.5% to over 20%. Incidence is likely to increase with the ageing population, increased survival of patients with comorbidities that are considered risk factors and improving sensitivity of imaging techniques. Use of clinical prediction scores, such as the Wells score, has assisted in clinical decision‐making and decreased unnecessary radiological investigations. However, imaging (i.e. computed tomography pulmonary angiography or ventilation‐perfusion scans) is still necessary for objective diagnosis. Anti‐coagulation remains the foundation of PE management. Haemodynamically unstable patients require thrombolysis unless absolutely contraindicated, while stable patients with right ventricular dysfunction or ischaemia should be aggressively anti‐coagulated. Stable patients with no right ventricular dysfunction can be discharged home early with anti‐coagulation and review. However, treatment should be case dependent with full consideration of the patient’s clinical state. Direct oral anti‐coagulants have become an alternative to vitamin K antagonists and are facilitating shorter hospital admissions. Additionally, duration of anti‐coagulation must be decided by considering any provoking factors, bleeding risk and comorbid state. Patients with truly unprovoked or idiopathic PE often require indefinite treatment, while in provoked cases it is typically 3 months with some patients requiring longer periods of 6–12 months.     

Wolff–Parkinson–White Syndrome and Acute Pulmonary Embolism: A Case Report

A 48‐year‐old woman admitted to the emerhency departments due to sudden onset of dyspnea. The diagnosis of pulmonary embolism (PE) was made and thrombolytic therapy was started. Baseline ECG showed signs of right ventricular overload and after thrombolysis, short PR, wide QRS, and delta wave indicating Wolf‐Parkinson‐White (WPW) pattern were appeared. In this case report, we discuss ECG of patient with PE mimicknig WPW.     

Absence of the fetal right pulmonary artery complicated with coarctation of the aorta: Prenatal and postnatal diagnosis

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiovascular malformation that can present as an isolated lesion or may be associated with other congenital heart malformations. Several studies have reported UAPA after birth. To our knowledge, the absence of the right pulmonary artery in the fetus has not been reported. Here, we report a rare case of fetal right pulmonary artery absence with aortic coarctation, which was confirmed by postpartum ultrasound and computed tomography angiography (CTA). Our case demonstrates that fetal echocardiography, especially the three‐vessel view, is beneficial for the prenatal diagnosis of pulmonary artery malformations.     

Single symptomatic colonic metastasis as first presentation of a pulmonary mucoepidermoid carcinoma

Metastatic lesions in the large bowel are rare. A case of single symptomatic colonic metastasis as first presentation of pulmonary mucoepidermoid carcinoma (MEC) is described. A 62‐year‐old male was admitted with constipation and rectal bleeding. Colonoscopy revealed extrinsic compression at sigmoid colon, and microscopy suggested a secondary origin. Subsequent chest computed tomography confirmed a right perihilar mass invading posterior mediastinum, carina and esophagus, and also a second consolidation on right lower lobe and ipsilateral mediastinal and subcarinal lymph nodes. After pathologic evaluation of a transthoracic biopsy specimen, the diagnosis of pulmonary high‐grade MEC, stage IV (T4, N3, M1b – 7th ed. TNM), was established. Chemotherapy with carboplatin and paclitaxel was initiated, and disease progression occurred after two cycles with increasing colonic mass. Palliative surgery was rejected because of advanced stage, and the patient was submitted to palliative pelvic radiotherapy. Second‐line chemotherapy with gemcitabine was initiated, but clinical status worsened and the patient died because of severe lung failure. Clinical, pathological and therapeutic aspects of this uncommon site of extrathoracic metastatic disease are discussed, emphasizing the important contribution of special stains and immunohistochemistry to the diagnosis.     

The Role of Echocardiography Doppler in Pulmonary Embolism

The purpose of this review is to make a critical analysis of selected literature about the role of echo-Doppler in suspected or proved venous thromboembolism and to address some issues about the potential use of echo-Doppler in specific situations in patients with pulmonary embolism (PE). Echo-Doppler is of great value in patients with suspected PE because many conditions that may be clinically mistaken for PE will be diagnosed. Echo-Doppler should not be used alone to rule out the diagnosis of PE because the ability of the technique in proving the diagnosis of PE in a nonselected population is limited. Echo-Doppler may be of value for diagnosis in selected subgroups of PE patients, mostly in scenarios like the emergency department or intensive care unit. Echo-Doppler is valuable in the hemodynamic assessment of patients with PE, making possible a strategy for risk stratification of in-hospital death in relation to the degree of right ventricle dysfunction at the time of diagnosis of acute PE. Echo-Doppler is useful for serial assessment of patients with established diagnosis of chronic thromboembolic pulmonary hypertension. Echo-Doppler may be useful in follow-up of patients after a diagnosis of acute PE to enable early identification of patients with persistent pulmonary hypertension / right ventricle dysfunction.     

Prognostic factors of mortality and 2-year survival analysis of systemic sclerosis with pulmonary arterial hypertension in Thailand

Aims: Pulmonary arterial hypertension (PAH) is a major complication and cause of death in systemic sclerosis (SSc). Our objective was to identify the predictive factors of mortality and the 2‐year survival rate among Thai sufferers of PAH‐SSc. Methods: An historical cohort study was performed among PAH‐SSc patients followed up at Srinagarind Hospital, Thailand, between January 2005 and December 2008. Kaplan‐Meier and Cox regression analyses were used to estimate the probability of survival and to assess the significant factors associated with death. Results: Pulmonary arterial hypertension was recognized in 60 patients using ECHO criteria, right ventricular systolic pressure (RVSP) > 35 mmHg. Two‐thirds of the patients were female, > 50 years of age, with the dSSc subtype. Twenty patients (33.3%) died: the mortality rate was 15.6% per 100 person‐years. The respective 1‐, 2‐, 3‐ and 4‐year survival rate was 86.1%, 71.3%, 64.6% and 53.9%. The majority (85%) died without any specific treatment for PAH. Using univariate analysis, the mortality risk was associated with: the WHO functional class (FC) III (HR = 27.82), visceral organ involvement (HR = 5.14), myositis (HR = 3.14), esophageal dysmotility (HR = 3.08) and pericarditis (HR = 2.84). Using Cox regression, the only predictor of death was FCIII. The causes of death in PAH‐SSc were related to PAH (60%), infection (30%) and acute renal failure (10%). Conclusion: Up to one‐third of Thai sufferers of PAH‐SSc died within 2 years of PAH diagnosis, without any specific treatment being given. Increased mortality risk was found in SSc patients who had FCIII and visceral organ involvement.     

Novel shunt modification with an adjustable stent‐embedded “fenestrated” septal occluder in a patient with pulmonary hypertension

A 60‐year‐old woman with progressive dyspnea and cyanosis, O2‐dependent pulmonary hypertension despite optimal medical therapy and remote atrial septostomy presented with worsening cyanosis and right‐to‐left shunting. The creation of a “fenestrated” ASD closure device with the insertion of a peripheral stent through an AMPLATZER? ASD closure device was deployed to minimize right to left shunting and allow for enlargement of the shunt if needed. This case demonstrates the benefit of diminishing a right to left shunt with a self‐fabricated fenestrated AMPLATZER device to improve symptoms in pulmonary hypertension patients with a pre‐existing ASD.     

Audit of the aetiology and prevalence of pulmonary hypertension in a tertiary hospital setting

Appropriate diagnosis and initiation of disease‐specific treatment is an important therapeutic goal in idiopathic pulmonary arterial hypertension. We evaluated the prevalence and aetiology of moderate‐to‐severe pulmonary hypertension in a cohort of patients referred for inpatient echocardiography, with significant pulmonary hypertension documented in 4.6%. Pulmonary hypertension complicating left heart disease was the most common aetiology, with idiopathic pulmonary arterial hypertension less frequent.     

Doubly committed subarterial ventricular septal defect: A risk factor for aortic distortion in patients considered for percutaneous pulmonary valve implantation?

Percutaneous pulmonary valve implantation has gradually become the first line strategy for re‐intervention for right ventricular outflow tract dysfunction during long‐term follow‐up after congenital cardiac surgery in many centers. We describe a case of a patient with double outlet right ventricle (Fallot's type) with a doubly committed subarterial ventricular septal defect, where the unique anatomy precluded percutaneous pulmonary valve implantation. © 2016 Wiley Periodicals, Inc.     

Prolonged survival after repeat resection of pulmonary metastasis from hepatocellular carcinoma

We report a patient in whom two pulmonary resections were performed for lung metastasis after hepatic resection of hepatocellular carcinoma (HCC). A 56‐year‐old Japanese man with an 8‐year history of chronic liver disease was admitted with elevated serum alpha‐fetoprotein (AFP) and a liver tumor that had been detected by ultrasonography. Computed tomography showed a 6‐cm tumor in the medial segment of the liver, and partial resection of the medial segment was performed. Thirty‐six months after the first operation, pulmonary resection was performed for a solitary metastasis in the left lung. Fifty‐one months after the second operation, a solitary metastatic tumor was detected in the right lung, without any evidence of recurrence or other metastatic foci, and thoracoscopic partial resection of the right lung was performed as the third operation. The patient is alive 36 months after the second pulmonary resection, has a normal AFP value, and shows no signs of recurrent or metastatic foci. Repeat pulmonary resection for metastasis from HCC resulted in long‐term survival in this patient.     

Echocardiographic diagnosis of partial anomalous pulmonary venous connection from right upper lobe to the coronary sinus

A 36-year-old woman was admitted because of an enlarged right heart. Echocardiographic examination revealed an abnormal vessel connecting to the dilated coronary sinus. The abnormal vessel traveled in the direction from the right axillary to the left epigastric region. Partial anomalous pulmonary venous connection (PAPVC) from the right upper lobe to the coronary sinus was initially considered as a possible diagnosis by echocardiography. At surgery, diagnosis of an isolated PAPVC of the right upper pulmonary vein to the coronary sinus was confirmed.     

The Diagnostic Accuracy of Doppler Echocardiography in Assessment of Pulmonary Artery Systolic Pressure: A Meta‐Analysis

Background: Transthoracic echocardiography is commonly used to estimate pulmonary arterial systolic pressure (PASP) and to diagnose pulmonary hypertension (PH). However, some recent studies have questioned the accuracy of Doppler echocardiography (DE) in the assessment of PASP. The present meta‐analysis was performed to estimate the accuracy, sensitivity, and specificity of DE in the assessment of PASP. Methods: A literature search and data extraction of English and non‐English articles reported from May 1984 to January 2009 was performed independently by 2 investigators using MEDLINE and EMBASE databases. Articles were included if they compared DE with right heart catheterization (RHC) in the assessment of PASP. Nine articles met our criteria and were included in our meta‐analysis. We conducted a meta‐analysis of the results of these articles using fixed‐ and random‐effect models to estimate the accuracy, sensitivity, and specificity of DE in the assessment of PASP. Results: The correlation between PASP estimated by DE and RHC ranged from (r = 0.65, P < 0.001) to (r = 0.97, P < 0.001). The pooled sensitivity, specificity, and accuracy of DE for the diagnosis of PH were 88% (95% confidence interval [CI], 84–92%), 56% (95% CI, 46–66%), and 63% (95% CI, 53–73%), respectively. Conclusion: DE is a useful noninvasive modality to screen for PH and can reliably determine whether PASP is normal, mildly elevated, or markedly elevated. However, abnormal results from DE need to be confirmed by RHC.     

Melatonin attenuates hypoxic pulmonary hypertension by inhibiting the inflammation and the proliferation of pulmonary arterial smooth muscle cells

Hypoxia‐induced inflammation and excessive proliferation of pulmonary artery smooth muscle cells (PASMCs) play important roles in the pathological process of hypoxic pulmonary hypertension (HPH). Melatonin possesses anti‐inflammatory and antiproliferative properties. However, the effect of melatonin on HPH remains unclear. In this study, adult Sprague–Dawley rats were exposed to intermittent chronic hypoxia for 4 wk to mimic a severe HPH condition. Hemodynamic and pulmonary pathomorphology data showed that chronic hypoxia significantly increased right ventricular systolic pressures (RVSP), weight of the right ventricle/left ventricle plus septum (RV/LV+S) ratio, and median width of pulmonary arterioles. Melatonin attenuated the elevation of RVSP, RV/LV+S, and mitigated the pulmonary vascular structure remodeling. Melatonin also suppressed the hypoxia‐induced high expression of proliferating cell nuclear antigen (PCNA), hypoxia‐inducible factor‐1α (HIF‐1α), and nuclear factor‐κB (NF‐κB). In vitro, melatonin concentration‐dependently inhibited the proliferation of PASMCs and the levels of phosphorylation of Akt and extracellular signal‐regulated kinases1/2 (ERK1/2) caused by hypoxia. These results suggested that melatonin might potentially prevent HPH via anti‐inflammatory and antiproliferative mechanisms.     

超声心动图诊断肺动脉高压157例分析

目的探讨超声心动图诊断肺动脉高压(PH)的临床价值。方法对157例确诊为PH患者给予超声心动图及CT、X线、肺动脉造影等检查,部分给予右心导管测量肺动脉收缩压(PASP),并与超声心动图测量肺动脉压值进行对比分析。结果本组157例患者中,经超声心动图确诊患者148例,确诊率94.3%。结合其他检查全部确诊。超声心动图误诊的9例中,6例为右肺动脉狭窄,3例为左肺动脉狭窄。肺动脉高压患者超声心动图检查主要表现为右心室肥厚增大,右心房扩大,肺动脉增宽,收缩期三尖瓣可见反流,反流频谱呈高速。30例患者右心导管测量PASP与超声心动图测量比较,差异无统计学意义(P>0.05)。结论超声心动图诊断PH具有准确率高、方便快捷、无创性、可重复性高等特点,且对病因的明确具有重要意义。     

A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease, with an incidence of <1 in 300 000 live births. We describe a rare case of a 23‐month‐old infant admitted for cough and fever. Echocardiography showed a branch of the left coronary artery originating from the pulmonary artery. The coronary computed tomographic angiography revealed anomalous origin of the left anterior descending (LAD) artery from the pulmonary artery with the left circumflex artery and right coronary artery arising normally from the aorta. The infant successfully underwent surgical reimplantation of the LAD to the ascending aorta. Our case emphasizes that echocardiography and computed tomographic angiography are valuable imaging modalities for making an accurate diagnosis and determining the precise surgical plan.     

The Role of Echocardiography in the Evaluation of Pulmonary Arterial Hypertension

The evaluation of pulmonary arterial hypertension (PAH) requires a multimodality approach that combines invasive and noninvasive imaging studies to ensure accurate diagnosis and classification. Given the complexity of the hemodynamic relationships between the left heart, pulmonary circulation, and right heart, the diagnosis of PAH is often a challenging task. Right heart catheterization is the gold standard for diagnosis, providing the hemodynamic information that defines the disease. Nonetheless, echocardiography continues to be a valuable tool in the approach to the patient with suspected PAH. Echocardiographic assessment generates a wealth of information about the response of the right heart to elevated pulmonary pressures and provides essential diagnostic and prognostic data to the clinician. Numerous measurements can be used to identify alterations in right heart morphology, pressure, and function; although each variable in isolation may have little utility, meaningful information is revealed when multiple parameters are considered together. In this article, we will review the echocardiographic measurements employed in assessment of the right heart and seek to clarify the role of echocardiography in the diagnostic workup of PAH.