Unusual presentation of patent ductus arteriosus in elderly patient

Abstract We presented a case of a 56-year-old man with giant pulmonary artery aneurysm caused by a misdiagnosed patent ductus arteriosus, severe multivalvular disease and active aortic valve endocarditis successfully treated by surgery. The correct diagnosis was missed despite preoperative diagnostics because the small patent ductus arteriosus was located at the distal part of common pulmonary trunk and a huge regurgitant signal overlapped its Doppler signal. Thorough evaluation of every patient, regardless of age, is necessary to recognize and treat this congenital anomaly.

Correlación del índice de perfusión con la repercusión del conducto arterioso en prematuros / Correlation of perfusion index with patent ductus arteriosus repercussion in premature newborns

Resumen Antecedentes: El conducto arterioso es una estructura necesaria en la circulación fetal, su persistencia puede provocar alteraciones hemodinámicas. El estándar de oro diagnóstico es la ecocardiografía, no siempre disponible. Las unidades de cuidados intensivos neonatales (UCIN) cuentan con oximetría de pulso, que mide el índice de perfusión (IP), el cual podría funcionar como auxiliar en el diagnóstico de persistencia del conducto arterioso hemodinámicamente significativo (PCAHs). Objetivo: Correlacionar el incremento del índice de perfusión (ΔIP) a las 24 y 72 h de vida extrauterina con PCAHs en recién nacidos prematuros de la UCIN de un hospital de segundo nivel. Material y métodos: Estudio de cohorte analítico prospectivo donde se incluyeron neonatos de 26 a 34 semanas de gestación, sin comorbilidades, a quienes se les realizó ecocardiograma y medición de IP en brazo y pierna a las 24 y 72 h. Se efectuó análisis bivariante con Y2/prueba exacta de Fisher y t de Student/U de Mann-Whitney, además correlación de Spearman y regresión lineal para predicción de valores. Resultados: Se incluyeron 39 prematuros. No se encontró diferencia significativa entre los pacientes sin y con PCAHs (mediana: 0.22 [0.06, 0.58] vs. 0.03 [–0.27, 0.2]; p = 0.09) a las 24 h de vida y tampoco a las 72 h de vida (mediana: 0.2 [0, 0.47] vs. 0.45 [–0.37, 0.76], p = 0.47). Se encontró una correlación positiva entre el diámetro del conducto arterioso (DCA) y el ΔIP (r: 0.78; IC 95%: 0.60-0.88; p = 0.01). La fórmula de predicción por regresión lineal se expresa así: DCA = 1.31 + (2.05 x ΔIP). Conclusiones: El IP no permite discriminar entre pacientes sin y con PCAHs. El ΔIP podría ser una herramienta para la monitorización del diámetro del conducto en neonatos después de las 72 h de vida.

Abstract Background: The ductus arteriosus is a necessary structure in fetal circulation, and its patency can produce hemodynamic alterations. The diagnostic gold standard is echocardiography, not always available. In the neonatal intensive care unit (NICU) they have pulse oximetry that measures perfusion index (PI), which could be used as a diagnostic tool in hemodynamic significant patent ductus arteriosus (HSPDA). Objective: To correlate the perfusion index increment (ΔPI) in 24 and 72 h after birth with HSPDA in premature newborns of NICU in a second level hospital. Materials and methods: This is an analytic prospective study which included neonates of 26-34 weeks of gestational age, without comorbidities, who underwent echocardiography and measurement of PI in arm and leg, 24 and 72 h after birth. We did bivariate analysis with Y2/exact Fisher test and Student t-test/Mann-Whitney U test, besides Spearman correlation and linear regression for value prediction. Results: We included 39 premature newborns. We did not find significant differences between patients without and with HSPDA (Median: 0.22 [0.06-0.58] vs. 0.03 [–0.27-0.2]; p = 0.09) at 24 h neither 72 h after birth (Median: 0.2 [0-0.47] vs. 0.45 [–0.37-0.76]; p = 0.47). We found a positive correlation between ductus arteriosus diameter (DAD) and ΔPI (r: 0.78; CI 95%: 0.6-0.88; p = 0.01). The prediction formula with linear regression is expressed this way: DAD = 1.31 + (2.05 x ΔIP). Conclusions: The PI doesn´t allow us to discriminate between patient without and with HSPDA. The ΔPI could be a tool for the monitorization of DAD in neonates 72 h after birth.

Persistencia del quinto arco aórtico asociado a persistencia del conducto arterioso / Persistent fifth aortic arch with patent ductus arteriosus

La persistencia del quinto arco aórtico es una rara anomalía congénita vascular que consiste en la presencia de una comunicación entre la aorta ascendente y la aorta descendente a través de un conducto arterial; se diagnostica de manera incidental. Informamos de un caso asociado a persistencia de conducto arterioso.

Persistent fifth aortic arch is a rare congenital vascular anomaly, with no clinical impact, so diagnosis is usually an incidental finding ocasionally associated with other congenital heart defects. We report a case of persistent fifth aortic arch associated with patent ductus arteriosus.

Custo-Efetividade Incremental do Tratamento Cirúrgico vs. Percutâneo da Persistência do Canal Arterial com o Amplatzer® Duct Occluder em Crianças: Revisão Sistemática / Incremental Cost-Effectiveness of Surgical vs. Percutaneous Treatment of Patent Ductus Arteriosus with the AmplatzerTM Duct Occluder in Children: A Systematic Review

Introdução: A persistência do canal arterial (PCA) é uma cardiopatia congênita relativamente comum e as alternativas para o tratamento de canais > 2,5 mm são a cirurgia ou a oclusão percutânea com próteses do tipo rolha. Essas últimas, apesar de consideradas o método de escolha, não estão previstas pelo Sistema Único de Saúde (SUS). Nosso objetivo foi comparar a razão de custo-efetividade incremental (RCEI) de ambas as estratégias. Métodos: Revisão sistemática em relação a desfechos clínicos e criação de modelo de decisão para avaliação da RCEI do Amplatzer® Duct Occluder (ADO) em comparação ao tratamento cirúrgico, para o fechamento da PCA. Os custos para ambos os métodos foram aqueles reembolsados pelo SUS em 2010, e o custo do conjunto (dispositivo + materiais de apoio) foi estimado em R$ 10.000,00. Foi considerado como limiar uma disposição para pagar equivalente a três vezes o Produto Interno Bruto brasileiro, resultando em R$ 57.000,00 por ano de vida salvo. Resultados: As duas técnicas foram seguras e eficazes, com menor morbidade e tempo de internação no fechamento percutâneo. A expectativa de vida ajustada foi similar nos dois grupos, sendo um pouco melhor para o ADO. O custo total foi calculado em R$ 8.507,00 para cirurgia e em R$ 11.000,00 para o ADO. A RCEI foi calculada em R$ 71.380,00 por ano de vida ganho. Uma análise de limiar demonstrou que a redução do valor do conjunto completo do ADO em R$ 492,65 traria a RCEI para o limiar aceitável para incorporação ao SUS nos dias de hoje. Conclusões: O tratamento percutâneo apresentou morbidade e tempo de internação menores, além de efetividade incremental semelhante àquela do tratamento cirúrgico. Com os...

Background: Patent ductus arteriosus (PDA) is a relatively common congenital heart disease and the alternatives for the treatment of PDA > 2.5 mm are surgery or percutaneous occlusion with plugs. The latter, although considered the method of choice, are not provided by the Brazilian National Health System (Sistema Único de Saúde - SUS). Our objective was to compare the incremental cost-effectiveness ratio (ICER) of both strategies. Methods: Systematic review of clinical outcomes and development of a decision-making algorithm to evaluate the ICER of AmplatzerTM Duct Occluder (ADO) vs. surgical treatment for the closure of PDA. Costs for both methods were calculated based on the reimbursement figures paid by the SUS in 2010 and the cost of the percutaneous kit (device + support materials) was estimated at R$ 10,000.00. We used as a threshold the willingness to pay the equivalent of three times the Brazilian Gross Domestic Product, i.e., R$ 57,000.00 per year of life saved. Results: Both techniques were safe and effective with less morbidity and shorter hospitalization time for percutaneous closure. Adjusted life expectancy was similar in both groups, and slightly better for the ADO group. Total cost was calculated as R$ 8,507 for surgery and R$ 11,000.00 for ADO. ICER was calculated as R$ 71,380.00 per year of life saved. A threshold analysis showed that a reduction of R$ 492.65 in the cost of the ADO kit would reduce the ICER to an acceptable value for the incorporation of this technology by the SUS. Conclusions: Percutaneous occlusion was associated with less morbidity and shorter hospital stay with similar incremental effectiveness when compared to the surgical treatment. With the direct costs used in this study and considering that the entire population with PDA is treated with the ADO, percutaneous occlusion was less cost-effective. However, a slight reduction in the costs of the percutaneous kit would result...

Haga su diagnóstico / Make your diagnosis

El diagnóstico es Ductus arterioso permeable (DAP)

The Timing of Surgical Ligation for Patent Ductus Arteriosus Is Associated with Neonatal Morbidity in Extremely Preterm Infants Born at 23-25 Weeks of Gestation

The purpose of this study was to evaluate prognostic factors associated with surgical ligation for patent ductus arteriosus (PDA) in extremely preterm infants born at the limits of viability. Ninety infants who were born at 23-25 weeks of gestation and who received surgical ligation were included and their cases were retrospectively reviewed. Infants were classified into two different groups: survivors with no major morbidity (N), and non-survivors or survivors with any major morbidity (M). Clinical characteristics were compared between the groups. Possible prognostic factors were derived from this comparison and further tested by logistic regression analysis. The mean gestational age and the mean birth weight of M were significantly lower than those of N. Notably, the mean postnatal age at time of ligation in N was significantly later than that of the other group (17+/-12 vs 11+/-8 days in N and M, respectively). An adjusted analysis showed that delayed ligation (>2 weeks) was uniquely associated with a significantly decreased risk for mortality or composite morbidity after surgical ligation (OR, 0.105; 95% CI, 0.012-0.928). In conclusion, delayed surgical ligation for PDA (>2 weeks) is associated with decreased mortality or morbidities in extremely preterm infants born at 23-25 weeks of gestation.

Mosaic Ring Chromosome 6 in an Infant with Significant Patent Ductus Arteriosus and Multiple Congenital Anomalies

The clinical features of ring chromosome 6 include central nervous system anomalies, growth retardation, facial dysmorphism and other congenital anomalies. Ring chromosome 6 occurs rarely and manifests as various phenotypes. We report the case of mosaic ring chromosome 6 by conventional karyotyping in a 7-day-old male infant diagnosed with a large patent ductus arteriosus (PDA) with hypoplasia of aortic valve and aortic arch. These have not been previously reported with ring chromosome 6. He recovered from heart failure symptoms after ligation of the PDA. He showed infantile failure to thrive and delayed milestone in a follow-up evaluation. To the best of our knowledge, this is the first report of a Korean individual with ring chromosome 6 and hemodynamically significant PDA.

Oclusão percutânea do canal arterial com molas de liberação controlada / Percutaneous occlusion of patent ductus arteriosus with controlled-release coils

INTRODUÇÃO: A oclusão percutânea do canal arterial tem sido realizada com eficácia e segurança, reservando-se o tratamento cirúrgico a situações específicas tecnicamente desfavoráveis à utilização da via percutânea, como a abordagem de prematuros. O dispositivo mola de liberação controlada configurou-se como técnica segura e de baixo custo. Apresentamos a experiência de nosso serviço com esse dispositivo, discutindo aspectos técnicos do implante e características do material utilizado. Método: No período de 2002 a 2009 foram encaminhados 90 pacientes para oclusão percutânea do canal arterial. Todos os pacientes foram diagnosticados em base clinicas, com confirmação por meio de ecocardiografia transtorácica. Foi realizado angiograma imediatamente após o procedimento para verificação da presença de fluxo residual e de possíveis complicações imediatas. Resultados: A idade variou de 4 meses a 36 anos (mediana de 3 anos e 4 meses). O diâmetro do canal arterial variou de 0,2 mm a 6 mm (média de 2,3 mm). Os tipos de canal arterial...

BACKGROUND: The percutaneous occlusion of patent ductus arteriosus is an effective and safe procedure and surgical intervention is restricted to specific unfavorable technical conditions, such as in premature infants. The controlledrelease coil has proven to be a low cost and safe method. We report our experience with this device, discussing technical aspects of the implant and coil characteristics. METHOD: From 2002 to 2009, 90 patients were referred for the percutaneous occlusion of patent ductus arteriosus. The size of the coil was based on the narrowest ductal luminal diameter. Diagnosis of all of the patients was based on clinical evaluation and transesophageal echocardiography. Aortic angiogram was performed immediately after the procedure to assess residual shunt and early complications. RESULTS: Age ranged from 4 months to 36 years (median: 3 years and 4 months). The patent ductus arteriosus diameter ranged from 0.2 mm to 6 mm (mean: 2.3 mm). According to Krichenko's morphological classification, the types of patent ductus arteriosus were divided as follows: type A, 80 (88.8%); type C, 4 (4.6%); and type E, 6 (6.6%). Success was achieved in 97.6% (81/83) of the cases. There was no evidence of hemolysis, endarteritis or vascular obstruction. We observed only one case of embolization where the device was retrieved back into the delivery catheter and repositioned successfully. CONCLUSION: This study showed that the controlled-release coil is safe and effective for the percutaneous occlusion of patent ductus arteriosus.

A Novel DHCR7 Mutation in a Smith-Lemli-Opitz Syndrome Infant Presenting with Neonatal Cholestasis

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive malformation syndrome caused by a defect in cholesterol biosynthesis. The incidence is very low in Asians and only one case has been reported in Korea thus far. Recently, we found an infant with neonatal cholestasis. He had microcephaly, ambiguous genitalia, cleft palate, syndactyly of toes, patent ductus arteriosus and hypertrophic pyloric stenosis. The serum cholesterol was decreased and serum 7-dehydrocholesterol was markedly elevated. Genetic analysis of the DHCR7 gene identified a novel missense mutation (Pro227Ser) as well as a known mutation (Gly303Arg) previously identified in a Japanese patient with SLOS. Although rare in Korea, SLOS should be considered in the differential diagnosis of neonatal cholestasis, especially in patients with multiple congenital anomalies and low serum cholesterol levels.

Tratamento endovascular da persistência do canal arterial em adulto / Patent ductus arteriosus: endovascular treatment in adult patient

A persistência do canal arterial (PCA) é uma anomalia relativamente freqüente e de simples correção. A correção envolve a ligadura do ducto com ou sem a sua secção. A anomalia em adultos pode provocar hipertensão pulmonar persistente e disfunção ventricular. A correção em adultos é controversa e de maior risco. Um caso de correção endovascular com acesso intra-abdominal da PCA em adulto é descrito.

The patent ductus arteriosus (PDA) is a common type of congenital heart defect and its correction is simple when performed early in life. Surgery is performed using stitches or clips. In adults, the anomaly can lead to pulmonary hypertension and ventricular dysfunction. Surgery in adults is controversial and high-risk. This report describes an alternative endovascular approach in an adult patient.

Patent ductus arteriosus in preterm neonates.

Failure of the ductus arteriosus to close within 48-96 hours of postnatal age results in a left to right shunt across the ductus and overloading of the pulmonary circulation. This is more likely to happen in premature neonates with respiratory distress syndrome. Deterioration in the respiratory status on day 3-4 in a ventilated neonate and unexplained metabolic acidosis may be the earliest indicators of a patent ductus arteriosus (PDA). Indomethacin is the main stay of medical management of PDA in preterm neonates. Guidelines for administration of indomethacin have been described in the protocol. Restricted fluid therapy may be beneficial in the prevention of PDA in preterm neonates. Presence of PDA in a term neonate should be investigated to rule out an underlying congenital heart disease.

Cardiopatia congênita no recém-nascido: da solicitação do pediatra à avaliação do cardiologista / Congenital heart diseases in the newborn: from the pediatrician's request to the cardiologist's evaluation

OBJETIVO: Analisar a importância dos sintomas, como motivo de interconsulta com o cardiologista pediátrico, no diagnóstico de cardiopatias congênitas (CC) em recém-nascidos (RN). MÉTODOS: Estudo prospectivo de RN vivos encaminhados para avaliação cardiológica, com realização de eletrocardiografia, radiografia de tórax e ecocardiografia. Solicitação de interconsulta mediante preenchimento de ficha de múltipla escolha, constando os sintomas e sinais sugestivos de CC. Persistência do canal arterial (PCA) sem repercussão clínica e/ou hemodinâmica não foi considerada cardiopatia. RESULTADOS: Entre 1999 e 2002, foram estudados 358 dentre 3.716 RN, demonstrando 49 casos de CC e 128 de PCA. A prevalência de CC foi de 13,2:1.000 RN. O principal motivo para interconsulta com o cardiologista foi sopro em 256 (72 por cento) dos RN, dentre os quais 39 (15 por cento) eram portadores de CC e 91 por cento dos 128 casos, de PCA. Em 14 (4 por cento) dos RN, o motivo de interconsulta foi cianose, dentre os quais 8 (57 por cento) eram portadores de CC. Insuficiência cardíaca foi o motivo de interconsulta em 37 (10 por cento) dos RN, dentre os quais 17 (46 por cento) eram portadores de CC. Arritmia, malformações congênitas associadas ou cromossomopatias foram os motivos de interconsulta em 14 por cento dos casos. CONCLUSÃO: O principal motivo da interconsulta foi ausculta de sopro. Apesar de cianose e insuficiência cardíaca serem pouco freqüentes como motivo de interconsulta, sua presença indicou alta probabilidade de diagnóstico de cardiopatia. A triagem pediátrica tem papel importante para o diagnóstico.

OBJECTIVE: To analyze the importance of symptoms as a reason for referral to pediatric cardiologists in the diagnosis of congenital heart diseases (CHD) in the newborn (NB). METHODS: Prospective study on live NB referred for cardiac evaluation, with performance of electrocardiogram, chest radiography and echocardiography. Cardiology consultation was requested by means of a multiple-choice form including signs and symptoms suggestive of CHD. Patent ductus arteriosus (PDA) without clinical and/or hemodynamic consequences was not considered a heart disease. RESULTS: From 1999 to 2002, 358 out of 3716 NB were studied, and 49 cases of CHD and 128 of PDA were found. The prevalence of CHD was 13.2:1000 NB. The main reason for referral to the cardiologist was heart murmur in 256 (72 percent) NB, of which 39 (15 percent) had CHD, and in 91 percent of the 128 cases of PDA. In 14 (4 percent) NB, the reason for referral was cyanosis, and eight of these patients (57 percent) had a CHD. Heart failure was the reason for referral in 37 (10 percent) NB, of whom 17 (46 percent) had CHD. Arrhythmia, associated congenital malformations, or chromosome disorders were the reasons for referral in 14 percent of the cases. CONCLUSION: The main reason for referral was detection of a heart murmur on cardiac auscultation. Although cyanosis and heart failure were uncommon reasons for referral, their presence indicated a high probability of the diagnosis of heart disease. Pediatric screening plays a key role in this diagnosis.

Stenting the patent arterial duct to increase pulmonary blood flow.

BACKGROUND: Use of surgically created aoropulmonary shunt is well-established for improving pulmonary blood flow in infants with critical reduction in pulmonary blood flow. Recently, stenting the patent ductus arteriosus has emerged as an alternative in selected infants with congenital heart disease and reduced pulmonary blood flow. METHODS AND RESULTS: We reviewed records of consecutive infants undergoing stenting of patent ductus arteriosus between August 2003 and October 2005 at our institution. Two of 12 patients underwent patent ductus arteriosus stenting to facilitate preparation of left ventricle for transposition with intact septum. We report the case selection, technique, immediate and short-term follow-up outcome in the remaining 10 patients [median age: 16 days (range 4-290 days): weight 2.7 kg (range 2-6 kg)] with reduced pulmonary blood flow who underwent stenting of patent ductus arteriosus as an alternative to conventional surgical aortopulmonary shunts. Five of the 6 newborns were prostaglandin-dependent and 4 had previously undergone guidewire perforation of the pulmonary valve (n=2) or balloon dilation (n=2). Successful stent implantation was accomplished in all with no major patient-related complication (median fluoroscopy time: 18.6 min; range: 7.7-72 min). The intensive care unit and hospital stays were prolonged in 3 patients because of sepsis (n=2) and pulmonary over-circulation with sepsis (n=1). On follow-up (median 5.5 months; range 1-19 months) all implanted stents were patent. One patient underwent re-dilation of the implanted stent for declining saturations. CONCLUSIONS: The immediate and short-term follow-up results of stenting of the patent arterial duct, as an alternative to the surgical aortopulmonary shunt in carefully selected newborns and infants is encouraging.

Persistência do canal arterial / Patent ductus arteriosus

A persistência do canal arterial é a manutenção, no período pós-natal, do conduto vascular musculoelástico, que comunica a circulação arterial sistêmica com a pulmonar, normalmente existente no desenvolvimento cardiológico fetal, originário do sexto arco aórtico embrionário. Este defeito congênito poderá ser diagnosticado em quatro situações clínicas distintas: 1- de forma isolada em infantes e crianças saudáveis, -2 como lesão isolada, em prematuros; -3 como achado ocasional em associação com outros defeitos intracardíacos; e -4 como defeito necessário, e de importância vital, em cardiopatias congênitas complexas.

Tratamiento de conducto arterioso permeable: Comparación de costos del cierre quirúrgico y transcateterismo en una institución pública / Treatment of patent ductus arteriosus: Comparison of costs between surgical and trans-catheter closures in a public institution

El costo del tratamiento transcateterismo del conducto arterioso permeable en comparación con el quirúrgico es un asunto controvertido en nuestro medio. El propósito de este estudio fue estimar y comparar los costos directos relacionados con ambos procedimientos. Método: Se incluyeron 57 pacientes tratados con intervencionismo y 26 con cirugía. Se obtuvo información sobre las características sociodemográficas, el número y tipo de exámenes de laboratorio y de gabinete, el tipo y duración de anestesia, la duración del procedimiento y la estancia hospitalaria y de terapia intensiva. Se construyó una matriz que integró los costos del sistema institucional de costos unitarios vigente. Resultados: Ambos grupos compartían características sociodemográficas. El diámetro del conducto fue mayor en el grupo quirúrgico (p<0.05). Tanto la estancia hospitalaria como el número de complicaciones post intervención fueron menores en los pacientes tratados con intervencionismo (p<0.05). El tratamiento con dispositivo Amplatzer® es más costoso que el tratamiento quirúrgico y ambos más costosos que el oclusor tipo resorte. En el tratamiento quirúrgico el 86.5% de los costos totales lo consumen la estancia hospitalaria, con el Amplatzer® este rubro fue del 36%, sin embargo el dispositivo representa el 40% del costo total. Conclusiones: No obstante el costo del tratamiento con dispositivo Amplatzer® es mayor que el quirúrgico, el cierre con oclusor representa ventajas con relación a menor estancia hospitalaria, consumo de recursos y número de complicaciones, lo que permite la optimización de los recursos hospitalarios.

The costs of transcatheter closure of patent ductus arteriosus in relation to the surgical closure still a controvertial issue in our hospitals. The aim of the study was compared the costs of both treatments. Methods: We included 57 patients treated with transcatheter occlusion and 26 underwent surgery. Information about laboratory tests, average in hospital days of stay, anesthesia type and duration, operating and hemodinamic room costs, was gather. A database containing the costs from the institution unitary costs system in force was designed. Results: sociodemographyc characteristics were similar in both groups. Ductus size was larger in patients treated with surgery (p<0.05). In hospital stay, as well as, the number of complications after the procedure were less in the patients treated with transcatheter occlusion (p<0.05). The closure with Amplatzer® device was more expensive than the surgical one, and both were more expensive than coil. With surgical treatment, 86.5% of the costs are due to in hospital stay, with the Amplatzer® this issues represented a 36%, however, the cost of the devices by itself represents a 40% of the total treatment cost. Conclusions: Even though total charges of Amplatzer® devices are more expensive than surgery, transcatheter occlusion represents advantages in relation to less in hospital stay, resources used and number of complications, which allows hospital resources optimization.