We present the case of a 51 years old female-patient, with severe dextroscoliosis, having like unique symptom progressive dyspnea. The blood samples reveals polycythemia, the radiological exam shows the opacification of 2/3 of the left thorax, the absence of the lung structure in the other 1/3, the deviation of the mediastinum, and dextroscoliosis; the computed tomography reveals the absence of the left lung artery and the left airways, compensatory hyperinflation of the right lung and dilatation of the trunk and right pulmonary artery; the bronchoscopy does not visualize the carina or the left main bronchus, typical for pulmonary agenesis. Echocardiography confirmed the absence of left pulmonary artery and shows mild pulmonary hypertension (systolic pressure in the pulmonary artery of 33 mmHg) with dilatation of the right cavities, but good cinetics. We face a case of pulmonary agenesis lately diagnosed, with modest functional cardiologic implications, limited therapeutic options and good survival, justified by the late appearance of the pulmonary hypertension of low severity and without worsening in time.

Download full-text PDF

Source

Publication Analysis

Top Keywords

pulmonary agenesis
12
pulmonary artery
12
agenesis diagnosed
8
absence left
8
pulmonary hypertension
8
pulmonary
7
left
5
[left pulmonary
4
diagnosed late]
4
late] case
4

Similar Publications

Disruption of developmental processes affecting the fetal lung leads to pulmonary hypoplasia. Pulmonary hypoplasia results from several conditions including congenital diaphragmatic hernia (CDH) and oligohydramnios. Both entities have high morbidity and mortality, and no effective therapy that fully restores normal lung development.

View Article and Find Full Text PDF

Unique imaging observations in an incidentally detected bilateral upper lobe agenesis.

BMJ Case Rep

November 2024

Radiology, Narayana Hrudayalaya Health City Bangalore, Bengaluru, Karnataka, India

Article Synopsis
  • - Bilateral upper lobe pulmonary agenesis is a rare birth defect typically discovered incidentally in adults, with non-specific and subtle findings on traditional x-rays.
  • - Diagnosis is often missed on regular radiography, but chest CT scans performed for other reasons can reveal this condition.
  • - Contrast-enhanced CT is the best method for diagnosing and detailing the features of pulmonary agenesis, and understanding related pulmonary anomalies is crucial in treating patients with congenital heart issues.
View Article and Find Full Text PDF

Purpose: Amniotic fluid stem cell extracellular vesicles (AFSC-EVs) hold regenerative potential to treat hypoplastic lungs secondary to congenital diaphragmatic hernia (CDH). This study aims to investigate sex-specific differences in pulmonary hypoplasia severity and responses to AFSC-EV administration in an experimental CDH mouse model.

Methods: C57BL/6J dams were fed with nitrofen + bisdiamine (left-sided CDH) or olive oil only (control) at embryonic day (E) 8.

View Article and Find Full Text PDF

Pulmonary agenesis (PA) is a rare developmental malformation, with a frequency of approximately 1 in 10-15,000 pregnancies. Unilateral PA is often associated with other congenital anomalies, whereas bilateral PA is fatal. Prenatal diagnosis is rare and is diagnosed more frequently in the postnatal period than in the prenatal period.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!