Systemic-pulmonary Collaterals Research Articles

Systemic-pulmonary collateral supply associated with clinical severity of chronic thromboembolic pulmonary hypertension: a study using intra-aortic computed tomography angiography

ObjectivesTo assess whether systemic-pulmonary collaterals are associated with clinical severity and extent of pulmonary perfusion defects in chronic thromboembolic pulmonary hypertension (CTEPH).MethodsThis prospective study was approved by a local ethics committee. Twenty-four patients diagnosed with inoperable CTEPH were enrolled between July 2014 and February 2017. Systemic-pulmonary collaterals were detected using pulmonary vascular enhancement on intra-aortic computed tomography (CT) angiography. The pulmonary enhancement parameters were calculated, including (1) Hounsfield unit differences (HUdiff) between pulmonary trunks and pulmonary arteries (PAs) or veins (PVs), namely HUdiff-PA and HUdiff-PV, on the segmental base; (2) the mean HUdiff-PA, mean HUdiff-PV, numbers of significantly enhanced PAs and PVs, on the patient base. Pulmonary perfusion defects were recorded and scored using the lung perfused blood volume (PBV) based on intravenous dual-energy CT (DECT) angiography. Pearson’s or Spearman’s correlation coefficients were used to evaluate correlations between the following: (1) segment-based intra-aortic CT and intravenous DECT parameters (2) patient-based intra-aortic CT parameters and clinical severity parameters or lung PBV scores. Statistical significance was set at p < 0.05.ResultsSegmental HUdiff-PV was correlated with the segmental perfusion defect score (r = 0.45, p < 0.01). The mean HUdiff-PV was correlated with the mean pulmonary arterial pressure (PAP) (r = 0.52, p < 0.01), cardiac output (rho = − 0.41, p = 0.05), and lung PBV score (rho = 0.43, p = 0.04). And the number of significantly enhanced PVs was correlated with the mean PAP (r = 0.54, p < 0.01), pulmonary vascular resistance (r = 0.54, p < 0.01), and lung PBV score (rho = 0.50, p = 0.01).ConclusionsPV enhancement measured by intra-aortic CT angiography reflects clinical severity and pulmonary perfusion defects in CTEPH.Key Points• Intra-aortic CT angiography demonstrated heterogeneous enhancement within the pulmonary vasculature, showing collaterals from the systemic arteries to the pulmonary circulation in CTEPH.• The degree of systemic-pulmonary collateral development was significantly correlated with the clinical severity of CTEPH and may be used to evaluate disease progression.• The distribution of systemic-pulmonary collaterals is positively correlated with perfusion defects in the lung segments in CTEPH.

A Particular Infantile Scimitar Syndrome Variant with Anomalous Systemic Arterial Supply-Inferior Vena Cava Fistula

Abstract Scimitar syndrome is a rare congenital cardiovascular malformation that includes a partially anomalous drainage of the pulmonary veins in the inferior vena cava, right pulmonary hypoplasia, and systemic-pulmonary collaterals originating from various segments of the aorta. We present a case of Scimitar syndrome with associated intracardiac lesions and a large arterial conduct supplying the right lung, originating from the abdominal aorta and draining in the inferior vena cava.

Effective Cardiac Index and Systemic-Pulmonary Collaterals Evaluated by Cardiac Magnetic Resonance Late After Fontan Palliation

The regulation of cardiac output in the Fontan circuit is not completely understood. Systemic-pulmonary collaterals (SPCs) are frequent in patients with univentricular heart, and their clinical significance and management remain controversial. The aims of our study were to identify factors associated with SPCs' flow at late follow-up after Fontan and evaluate the relation between SPCs flow (QSPCs) and the effective cardiac index (CI). From our cardiac magnetic resonance database, we identified all Fontan patients with a complete set of flow measurements allowing calculation of QSPCs and effective CI. QSPCs was calculated as (left pulmonary veins flow+ right pulmonary veins flow)- (right pulmonary artery flow+ left pulmonary artery flow). Effective CI was calculated as (Aortic flow (QAo)- QSPCs)/BSA. Medical, surgical history, and clinical status were recorded. Sixty-four post-Fontan patients (36male; mean age 19 ± 10years) were included in the study. Median QSPCs was 0.7L/min/m2 (interquartile [IQ] range 0.386-0.983) accounting for a median of 21% (IQ range 13-28) of aortic flow. The effective CI in our population was 2.4 ± 0.6L/min/m2. QSPCs inversely correlate with left pulmonary artery area (r=-0.37, p= 0.004) and total antegrade pulmonary flow (r=-0.32, p= 0.01). QSPCs correlate with indexed aortic flow (r= 0.6, p <0.001) and inversely correlate with effective CI (r=-0.39, p= 0.002). Effective CI inversely correlates with age at study and age at the Fontan palliation (r=-0.35, p= 0.005, and r= -0.29, p= 0.02, respectively) and positively with ventricular ejection fraction (r= 0.3, p= 0.01). In conclusion, SPCs arecommon in Fontan patients, correlate inversely with effective CI, and are associatedwith a reduced antegrade pulmonary flow. In cardiac magnetic resonance evaluation of post-Fontan patients, effective CI should be taken into account rather than the total CI.

English

Valvar pulmonary atresia is a rare congenital anomaly usually associated with ventricular septal defects or intact ventricular septum. The patent ductus arteriosus (56%) and systemic pulmonary collaterals maintain pulmonary blood flow. Presentation beyond childhood is unusual, as there is usually high morbidity in the first year of life. The two index cases highlight the rarity of adolescents with valvar pulmonary atresia and focusses on the transthoracic echocardiographic features, whose diagnoses provide definitive and curative options with adequate size pulmonary dimensions. Options available are transcatheter interventional radiofrequency pulmonary valvotomy, which is both diagnostic and therapeutic, and intraoperative valve replacement, thus eliminating the need for right ventricle pulmonary artery external valved conduit during corrective surgery. They are the first cases of adolescents with isolated cardiac anomaly of valvar pulmonary atresia, documented in Afro-Caribbeans.

Anaesthetic management for drainage of frontoparietal abscess in a patient of uncorrected Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, characterised by aortic overriding, right ventricular hypertrophy, pulmonary stenosis (PS) and ventricular septal defect (VSD). When left untreated, survival to adulthood is rare.[1] Brain abscesses can occur in them because of paradoxical embolism and absence of pulmonary phagocytic clearance of pathogens. There are few case reports of uncorrected TOF surviving to adulthood and then presenting for non-cardiac surgeries.[1,2,3] We present a case of drainage of a large left fronto-parietal abscess under general anaesthesia, in a patient of uncorrected TOF.

THE RECRUITMENT OF SYSTEMIC TO PULMONARY COLLATERALS IN CHRONIC AND SEVERE PULMONARY HYPERTENSION AS AN ADAPTIVE MECHANISM: DO WE TREAT THESE COLLATERALS IN REALITY?

Bronchial arteries (BAs) form a pre-alveolar anastomotic network with pulmonary arterioles which supply a flow of 0.5-2% of the cardiac output. In conditions such as pulmonary arterial obstruction (PAO) and pulmonary hypertension (PH), recruitment of systemic-pulmonary collaterals (SPC) via enlarged

Uso do Sistema Manom&amp;#233;trico Miniaturizado Pressure-Wire em Cardiopatias Estruturais Cong&amp;#234;nitas e Adquiridas

Introdução: Historicamente, o registro de pressões intravasculares contribuiu para o desenvolvimento da cardiologia intervencionista. Embora atualmente novos recursos de imagem sejam preponderantes, a medida acurada de pressões intravasculares ainda é essencial em muitos contextos diagnósticos e de aferição de métodos terapêuticos intervencionistas. Descrevemos a utilização do sistema manométrico miniaturizado (pressure-wire) para obtenção de curvas pressóricas em cardiopatias estruturais congênitas e adquiridas. Métodos: Foram utilizados os sistemas RADI Analyzer® Xpress (St. Jude Medical Inc., St. Paul, Minnesota, Estados Unidos) e PressureWire® Certus durante procedimentos com cateteres diagnósticos 5 F realizados sob anestesia geral e suporte ventilatório em crianças. Traçados manométricos foram obtidos simultaneamente com o pressure-wire e o cateter 5 F em pacientes para os quais era imperativa a decisão terapêutica com base em análise das pressões intravasculares e nos quais não era possível obtê-las acuradamente pelos métodos convencionais. Resultados: O pressure-wire foi utilizado para obtenção de pressões intravasculares pulmonares em pacientes com colaterais sistêmico-pulmonares, com estenoses angiográficas ou não, e com variadas cardiopatias estruturais, na avaliação de estenoses de ramos pulmonares, na localização de estenoses de condutos cirúrgicos (por exemplo: após cirurgia de Rastelli), shunts cirúrgicos (como de Blalock-Taussig) e na avaliação pré-operatória de derivações cavo-pulmonares. O procedimento foi realizado com segurança, tendo sido obtidos traçados manométricos com qualidade adequada. Conclusões: O sistema manométrico miniaturizado está consagrado no cenário da cardiologia intervencionista, enquanto modalidade diagnóstica complementar para avaliação funcional de lesões coronárias. Esse sistema também constitui método diagnóstico complementar em diversas cardiopatias estruturais, congênitas e adquiridas.

Case of Paradoxical Embolic Transient Ischemic Attack from an Unusual Extra Cardiac Shunt Detected on a Contrast-Enhanced Echocardiogram

Paradoxical embolism due to extracardiac right to left shunts (RLSs) manifesting as stroke remains anecdotal. We describe a case of 63-year-old female who presented with a transient ischemic attack and at agitated saline contrast echocardiogram was found to have an unusual type of an extracardiac RLS. Further evaluation leads to diagnosis of superior vena cava (SVC) thrombosis from a prior indwelling central venous catheter. The SVC thrombosis induced systemic-pulmonary venous collaterals were responsible for this unusual extracardiac RLS and paradoxical transient ischemic attack. We discuss the diagnosis and management of this rare clinical case.

Cardiac magnetic resonance versus routine cardiac catheterization before bidirectional Glenn anastomosis: Long-term follow-up of a prospective randomized trial

A previously published randomized clinical trial comparing cardiac magnetic resonance (CMR) versus routine catheterization in patients with functional single ventricle before bidirectional Glenn (BDG) operation demonstrated similar short-term post-BDG outcomes. We sought to assess late outcomes in this cohort to ascertain any long-term effects of this evaluation strategy. Retrospective review of enrolled patients through most recent follow-up was performed on all 82 patients in the original cohort, at a median age of 8.8 years. Of these, 76 (93%) underwent Fontan operation; 2 died before Fontan. Baseline demographics, anatomic factors, and age at BDG did not differ between those randomized to CMR versus catheterization. Although pre-BDG CMR patients were younger at Fontan (2.4 vs 2.7 years; P=.02), baseline weight, body surface area, oxygen saturation, ventricular function, and degree of atrioventricular valve regurgitation were similar. Catheterization before Fontan (n=76) demonstrated similar hemodynamic parameters including pulmonary vascular resistance and mean pulmonary artery, atrial, and ventricular end-diastolic pressures. CMR patients had comparable rates of transcatheter interventions (71% vs 79%; P=.6), including coil occlusion of systemic-pulmonary collaterals (66% vs 61%; P=.29). At Fontan surgery, short-term complications, hospital length of stay, and the percent meeting a 5-part definition of successful Fontan operation were not different (71% vs 55%; P=.23). Pre-BDG CMR and catheterization groups had equivalent clinical and hemodynamic profiles before Fontan and similar post-Fontan outcomes at a median follow-up of 8 years after BDG. For selected patients, a pre-BDG evaluation with CMR is an acceptable alternative to catheterization.

Plastic Bronchitis After Extracardiac Fontan Operation

We discuss 4 cases of plastic bronchitis (PB) after Fontan procedure observed at our Institution, with the aim to identify an effective treatment and possible factors favoring the onset of PB. Plastic bronchitis is an uncommon disease characterized by recurrent formation of large pale bronchial casts obstructing the tracheobronchial tree. The treatment includes inhaled or systemic steroids, aerosolized mucolytics, bronchoscopic lavage, direct bronchoscopic extraction and, in few reports, aerosolized fibrinolytic and pulmonary vasodilators. Four cases of PB after Fontan procedure occurred in our center from January 2008 to January 2012. All patients showed preserved ventricle function and underwent embolization of systemic-pulmonary collaterals prior to a Fontan procedure and all had chylothorax in the perioperative period. Two patients died; 1 of massive thrombosis of the superior vena cava and right pulmonary artery and the other of acute asphyxiation and refractory cardiac failure. The exact causes of PB in Fontan patients remain unknown. An early diagnosis and a multiple therapy with steroids, mucolytics, pulmonary vasodilators, and aerosolized tissue plasminogen activator may be effective.

Truncus arteriosus operado aos 28 anos: importância do diagnóstico diferencial

This is the case of 28 year-old adult with suspected congenital heart disease since birth, not treated in childhood at the his family's choice. At 27 years old, he was diagnosed with pulmonary atresia with ventricular septal defect and systemic-pulmonary collaterals, where surgery was contraindicated. A new review in our department showed that it was an atypical form of truncus arteriosus. The fact that a common arterial trunk with left-right shunt was viewed by echocardiography was a crucial fact for the indication of new catheterization, opening the prospect of surgical correction. Currently, the patient is well, with 7 years of postoperative outcome.

Experiência clínica com a utilização de plugs vasculares Amplatzer®

INTRODUÇÃO: Os plugs vasculares Amplatzer® I e II (PVAs I e II) são dispositivos de nitinol autoexpansíveis e de baixo perfil desenvolvidos para oclusão de estruturas vasculares. Relatamos nossa experiência com esses dispositivos em dois centros brasileiros de referência. MÉTODOS: Estudo retrospectivo dos pacientes que se submeteram a procedimentos de oclusão de estruturas vasculares diversas com PVAs desde 2005. Foram utilizadas próteses 30% a 50% maiores que o vaso-alvo, implantadas sob anestesia geral por via venosa femoral e jugular interna ou arterial femoral e braquial por cateteres terapêuticos ou bainhas longas 5 F a 8 F. RESULTADOS: Foram identificados 14 pacientes (50% do sexo masculino) com mediana de idade de 5 anos (11 meses a 70 anos) e mediana de peso de 15 kg (8 kg a 67 kg). Foram utilizados 17 plugs vasculares, sendo 2 PVAs II e 15 PVAs I, com diâmetros de 4 mm a 16 mm. Em 3 pacientes existiam dois vasos para oclusão, sendo utilizados 2 PVAs em vasos diferentes. Em 3 pacientes com fístulas coronárias houve oclusão completa dos vasos após 24 horas. Em 2 pacientes com anastomose de Blalock-Taussig houve oclusão completa da anastomose, em um deles com auxílio de molas adicionais. Em 3 pacientes com fístulas venovenosas no pós-operatório de Glenn ou Fontan houve oclusão completa dos vasos e melhora da saturação, em um deles com auxílio de molas adicionais. Outro caso de fístula venovenosa apresentava fluxo residual discreto no laboratório, com oclusão na monitorização ecocardiográfica em 24 horas. Em 2 pacientes com fístula arteriovenosa pulmonar foram utilizados, além dos PVAs, outras próteses Amplatzer e molas de Gianturco, permanecendo mínimo fluxo residual em ambos os casos. Em 2 pacientes com colaterais sistêmicopulmonares no pós-operatório de atresia pulmonar com comunicação interventricular, houve oclusão completa com auxílio de molas, sendo um imediato e outro em 24 horas. Em um paciente com síndrome da cimitarra a colateral sistêmica foi ocluída totalmente. Não houve episódios de embolização ou mortalidade. CONCLUSÕES: Os PVAs mostraram-se adequados e versáteis para oclusão de diferentes estruturas vasculares, com facilidade de implante e bons índices de oclusão. Em alguns casos, houve necessidade de uso de outros dispositivos. Nesses casos, os PVAs funcionam bem para ancorar os dispositivos adicionais.

HEPARIN AS A RISK FACTOR FOR PERIGRAFT SEROMA COMPLICATING THE MODIFIED BLALOCK-TAUSSIG SHUNT

Objective: The purpose of this study was to determine the risk factors associated with the occurrence of perigraft seromas complicating systemic-to-pulmonary polytetrafluoroethylene grafts. Methods: Clinical and perioperative variables were reexamined, blinded for the outcome variable perigraft seroma, in 60 patients undergoing 67 consecutive graft procedures in a 3.5-year period. Results: Eight cases of perigraft seroma were diagnosed in six patients. Univariate analysis revealed age ( p = 0.02), a diagnosis of pulmonary atresia with ventricular septal defect and systemic–pulmonary collaterals ( p = 0.001), reimplantation of collaterals during the procedure ( p < 0.001), and intravenous heparin administered after operation ( p < 0.0001) as risk factors for symptomatic perigraft seroma. Multivariable analysis defined heparin as the only significant factor associated with symptomatic perigraft seroma. Consolidation of the upper lobe on chest radiograph, ipsilateral to the shunt, directly after operation ( p = 0.01), but especially 8 to 10 days after operation ( p < 0.0001), or the need for prolonged drainage of pleural fluid ( p < 0.0001) were correlated with the occurrence of perigraft seroma. Perigraft seroma led to four early rethoracotomies in three patients and to accelerated corrective surgery in three cases. Consolidation and absent perfusion of lung segments persisted in two patients. Conclusions: Our data suggest that the use of heparin leads to an increased risk of perigraft seroma, complicating systemic–pulmonary polytetrafluoroethylene grafts. Prolonged pleural drainage and/or postoperative consolidation of the upper lobe indicate the development of symptomatic perigraft seroma. Treatment is controversial and results are unpredictable. Expectative management seems to be justified so long as permitted by the clinical condition. (J Thorac Cardiovasc Surg 1998;116:286-93)

Clinical Experience of Transcatheter Coil Embolization in Children

Background:Transcatheter coil embolization has been described as a method of nonsurgical closure of variable pathologic vascular structures. This study was aimed to evaluate the outcome of transcatheter coil embolization in variable clinical conditions. Methods and Results:We collected data from patients’ medical record andtheir cardiac angiography films. From January 1995 to June 1997, coil embolization was attempted in 51 patients who were 38 patients with systemic-pulmonary collaterals (5 patients have venous collaterals, too), six patients with venous collaterals, nine patients with patent ductus arteriosus (PDA), one patient with Blalok-Taussig shunt (BT shunt), one patient with coronary-right atrial fistula and one patient with coronary-right ventricular fistula. In 38 patients with systemic-pulmonary collaterals, 123 coils were inserted to 70 collaterals, therefore mean 1.79±0.77 coils were inserted to one collateral. The results were complete occlusions (74%), incomplete occlusions (21%), and partial occlusions (4%). In six patients with venous collaterals, the outcomes were complete occlusions (50%) and incomplete occlusions (50%). In a patient with BT shunt, hemolytic anemia occurred in 1st attempt and in 2nd attempt, shunt was incompletely occluded and one coil was carried away and embolized the peripheral pulmonary artery. In nine patients with PDA, ten cases of transcatheter coil embolization was executed. Mean minimum ductal diameter was 2.1±0.85 mm. The results were initial occlusion (30%), occlusion within one month(66%), and occlusion within one year (75%). Left pulmonary artery stenosis owing to coil insertion was not found. In one case of coil malposition, retrieval and reinsertion of coil was successful. In two patients who have coronary artery fistula, coil embolization was successfully executed without any complications. Conclusions:Transcatheter coil embolization executed in variable clinical conditions without significant complications. It was effective and safe nonsurgical method. (Korean Circulation J 1998;28(4):691-699)

Hemodynamic reactions in the acute phase of massive pulmonary embolism

Massive pulmonary embolism is accompanied by the differences between a decrease in systemic and peripheral blood flow and imbalanced blood ejection from the ventricles. The results of the study suggest an association of these hemodynamic responses with redistribution of blood outflows from systemic veins whereby the relative proportion of blood outflowing to the right atrium decreases and that of blood outflowing to the left atrium increases via systemic-pulmonary collaterals.

Tratamento cirúrgico da ectopia cordis: relato de três casos e revisão da literatura

Entre 1985 e 1990, tres neonatos portadores de ectopia cordis (EC) foram admitidos no InCor - FMUSP; dois com defeito do tipo toraco-abdominal e um do tipo toracico. Todas as criancas tinham anomalias cardiacas associadas: atresia tricuspide tipo I-A, ausencia de arterias pulmonares centrais e colaterais sistemico-pulmonares (um paciente); comunicacao interventricular (CIV) e comunicacao interatrial (CIA) (um paciente); CIA (um paciente). Uma crianca nao foi operada, devido a infeccao e laceracao da arteria pulmonar, indo a obito por sangramento. As duas outras foram operadas, na tentativa de se recobrir o coracao com pele; uma foi a obito no 1o dia de pos-operatorio, por baixo debito (BD); a outra teve evolucao mais longa, indo a obito no 141o dia de pos-operatorio, causado por insuficiencia respiratoria. A longa evolucao desse ultimo paciente deveu-se a um defeito cardiaco de pouca repercussao hemodinâmica (CIA) e a colaboracao de um cirurgiao plastico na equipe cirurgica. Este trabalho relata esses tres casos e faz uma suscinta revisao da literatura.

Surgical management of pulmonary atresia and ventricular septal defect with major systemicpulmonary collaterals

Surgical management of pulmonary atresia and ventricular septal defect with major systemicpulmonary collaterals

Correction of pulmonary atresia with ventricular septal defect in the absence of the pulmonary trunk and the central pulmonary arteries (so-called truncus type IV)

A child with pulmonary atresia and ventricular septal defect and no extraparenchymal pulmonary arteries had all the bronchopulmonary arterial segments connected to naturally occurring systemic-pulmonary collaterals. A three-staged surgical correction was performed. At the first and second stages, the arteries of each hilus were interconnected with synthetic arteries. At the third stage, the ventricular septal defect was closed, and continuity between the right ventricle and the bilateral pulmonary circulation was established with a valved conduit giving rise to a side arm. Postoperative evolution was good, with acceptable postrepair per right ventricular--left ventricular pressure ratio.

Novas técnicas cirúrgicas para o tratamento da atresia pulmonar com comunicação interventricular e anomalias de artérias pulmonares incluindo o assim chamado truncus tipo IV

Entre janeiro de 1975 e outubro de 1986, 42 pacientes com atresia pulmonar e comunicacao interventricular, com idade entre 2 e 18 anos, foram submetidos a correcao parcial, ou total. Foram divididos em: tipo A) com todos os segmentos broncopulmonares conectados as arterias pulmonares (AP's), 34 pacientes; tipo B) com alguns dos segmentos broncopulmonares conectados as AP's, 6 pacientes; tipo C) com todos os segmentos broncopulmonares conectados as colaterais sistemico-pulmonares, 2 pacientes. A correcao foi planejada em uma a tres etapas. No tipo A, 17 foram corrigidos em uma etapa, com tres obitos; em 9, na primeira etapa, as AP's foram reconstruidas e o Blalock (BT), realizado, tendo ocorrido um obito. Em 2, a segunda etapa de correcao total foi realizada, sem obitos. No tipo B, a primeira etapa de unificacao das colaterais intra ou extra-hilares foi realizada em 6 casos, sem obitos; em 2, a segunda etapa da correcao total foi realizada, com um obito. No tipo C, 2 pacientes foram operados; 1 em tres etapas; a primeira constou de construcao de segmento arterial intermediario entre as arterias lobares e o BT; a segunda compreendeu unificacao das colaterais contralaterais e a terceira, restabelecimento da continuidade ventriculo direito - circulacao pulmonar; o paciente teve boa evolucao. No segundo caso, a correcao foi realizada apos somente uma intervencao previa. A evolucao foi satisfatoria. Estudos hemodinâmicos seriados foram realizados em 32 pacientes. As tecnicas propostas permitem obter condicoes para correcao total com adequada relacao pos-operatoria das pressoes ventriculo direito - ventriculo esquerdo.

Surgical Correction of Pulmonary Atresia and Ventricular Septal Defect with Large Systemic-Pulmonary Collaterals

A two-stage surgical procedure was performed in a young adult with pulmonary atresia and a ventricular septal defect with upper lobe pulmonary artery flow from confluent central pulmonary arteries and lower lobe pulmonary blood flow originating from two large systemic–pulmonary collaterals. Initially a Dacron γ graft was anastomosed between the ascending aorta and the collaterals and a graft to the left pulmonary artery. At the second operation, continuity was established between the right ventricle and the γ graft by using a valved Dacron conduit and the ventricular septal defect was closed.